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224 Cards in this Set
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RBC production problem on CBC?
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RBC production problem on CBC? Low Recticulocyte count. For both Microcytic and Macrocytic anemia.
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Causes of Microcytic anemia?
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Causes of Microcytic anemia? Low MCV. Low Reticulocyte. TICS - 1 Thalassemia, 2 Iron Deficiency, 3 Anemia of Chronic disease, 4 Sideroblaastic anemia.
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Causes of Macrocytic anemia?
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Causes of Macrocytic anemia? High MCV. Low Reticulocyte. 1 B12 and Folate Deficiency, 2 Sideroblastic anemia, 3 Alcoholism, 4 Antimetabolite medications (Azathioprine, 6-Mercaptopurine, or Hydroxyurea), 5 Liver disease or Hypothyroidism, 6 Medications (Zidovudine, or Phenytoin), 7 Myelodysplastic syndrome (MDS)
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What does Blood loss and Hemolysis change in CBC?
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What does Blood loss and Hemolysis change in CBC? Blood loss and Hemolysis Raise Reticulocytes count.
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What to look for in Microcytic and Macrocytic anemia?
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What to look for in Microcytic and Macrocytic anemia? MCV Low (Microcytic anemia), MCV High (Macrocytic)
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Anemia Tx?
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Anemia Tx? Severe - pRBC (Transfuse). 1 Symptomatic, 2 Hematocrit Very Low (HCT NL 40. HCT 25-30 - Very Low), 3 Elderly, 4 Heart Disease - Tx - Transfuse.
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Normal HCT?
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Normal HCT? Hematocrit NL 40.
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What is Whole Blood?
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What is Whole Blood? Whole Blood has pRBC and FFP (Fresh Frozen Plasma).
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What is FFP?
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What is FFP? Fresh Frozen Plasma has Clotting Factor - Tx Increase PT, PTT, INR, BT.
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What is used in Plasmapheresis?
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What is used in Plasmapheresis? FFP is used
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FFP is not a choice for what disease?
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FFP is not a choice for what disease? Hemophilia A or B or von Willebrand disease.
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What is Cryoprecipitate?
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What is Cryoprecipitate? Cryoprecipitate is Never used first for anything. Cryoprecipitate replace Fibrinogen. Some utility is DIC - High amounts of clotting factors in smaller plasma volume.
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What is hypersegmented neutrophils?
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What is hypersegmented neutrophils? Neutrophil with Six or more lobes. It is present in 1 B12 deficiency, 2 Folate deficiency, 3 Antimetabolite medications (1 Azathioprine, 2 6-Mercaptopurine, 3 Hydroxyurea)
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Target cells. Most common Dx?
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Target cells. Most common Dx? Thalassemia.
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Reticulocyte count Normal? Abnormal?
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Reticulocyte count Normal? Reticulocyte count Normal 1-1.5 perc. Retic High - Hemolytic Anemia. Retic Low - Microcytic anemia - TICS.
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In Anemia of Chronic Disease, what disease is most definite?
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In Anemia of Chronic Disease, what disease is most definite? Renal Failure with Deficiency of Erythropoietin.
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Anemia. Alcoholic. Dx?
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Anemia. Alcoholic. Dx? Sideroblastic anemia.
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Anemia. Low Ferritin. Dx?
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Anemia. Low Ferritin. Dx? Iron Deficiency. Ferritin is Stored Iron. Ferritin is Acute phase reactant.
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Anemia. High Iron. Dx?
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Anemia. High Iron. Dx? Sideroblastic anemia.
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Anemia. Normal Iron Studies. Dx?
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Anemia. Normal Iron Studies. Dx? Thalassemia.
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Anemia. Increased TIBC. Dx?
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Anemia. Increased TIBC. Dx? Iron Deficiency
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Anemia. Low TIBC. Dx?
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Anemia. Low TIBC. Dx? Anemia of Chronic disease.
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Anemia. Increased Iron. Dx?
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Anemia. Increased Iron. Dx? Sideroblastic anemia.
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Anemia. Increased RDW. Dx?
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Anemia. Increased RDW. Dx? Iron Deficiency. RDW Normal Less than 15 perc.
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Iron Deficiency anemia. Lx Initial? Most Accurate?
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Iron Deficiency anemia. Lx Initial? Iron Studies. Most Accurate? Bone Marrow Biopsy for stainable iron - Decreased.
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Prussian blue test. Dx?
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Prussian blue test. Dx? 1 Sideroblastic anemia - Prussian Blue staining for Ringed Sideroblasts - Most accurate Lx. Basophilic stippling can occur in any cause of sideroblastic anemia. 2 Myelodysplastic Syndrome (MDS)
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Sideroblastic anemia. Most accurate Lx?
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Prussian blue test. Dx? Sideroblastic anemia - Prussian Blue staining for Ringed Sideroblasts - Most accurate Lx. Basophilic stippling can occur in any cause of sideroblastic anemia.
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Alpha Thalassemia. Beta Thalassemia. Defect?
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Alpha Thalassemia. Beta Thalassemia. Defect? Alpha Thalassemia has Alpha unit defect. Beta has Beta unit defect.
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Cooley anemia. Tx?
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Beta Thalassemia major (Cooley anemia) managed with Chronic Transfusion lifelong. Iron overload managed with Deferasirox (oral chelator). Deferoxamine is parenteral version of iron chelator.
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Iron Deficiency anemia Tx?
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Iron Deficiency anemia Tx? Replace iron with Oral Ferrous sulfate. Occassionally, Intramuscular iron.
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Anemia of Chronic Disease. Tx?
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Anemia of Chronic Disease. Tx? Correct underlying disease. Only anemia associate with End-stage renal failure - Tx - Erythropoietin replacement.
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Sideroblastic anemia. Tx?
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Sideroblastic anemia. Tx? Correct the cause. Some pt respond to Vitamin B6 or Pyridoxine replacement.
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73 yo man with fatigue progressively getting worse over last several month. SOB when walks up one flight of stairs. Drinks 4 vodka martinis a day. Complains of numbness and tingling in his feet. Physical examination has decreased sensation of his feet. Hematocrit is 28 perc and MCV is 114. Next step?
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73 yo man with fatigue progressively getting worse over last several month. SOB when walks up one flight of stairs. Drinks 4 vodka martinis a day. Complains of numbness and tingling in his feet. Physical examination has decreased sensation of his feet. Hematocrit is 28 perc and MCV is 114. Next step? Peripheral smear. B12 or folate deficiency, direct alcohol effect on bone marrow, or liver diseaes cause macrocytic anemia. First step is a Peripheral smear - detects Hypersegmented neutrophils. Once hypersegmented neutrophils are seen, get B12 and folate levels.
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Vitamin B12 deficiency causes?
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Vitamin B12 deficiency causes? 1 Pernicious anemia, 2 Pancreatic insufficiency, 3 Dietary deficiency (vegan or strict vegetarian), 4 Crohn disease damaging terminal ileum, 5 Blind loop syndrome (gastrectomy or gastric bypass for weight loss), 6 Diphyllobothrium latum.
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Causes of Folate deficiency?
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Folate deficiency causes? 1 Dietary deficiency (goat milk has no folate and limited iron and B12), 2 Psoriasis and skin loss or turnover, 3 Drugs (Phenytoin, Sulfa).
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What does Celiac disease cause?
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What does Celiac disease cause? B12, Folate, and Iron deficiency.
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Methotrexate use in rheumatoid arthritis. What disease?
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Methotrexate use in rheumatoid arthritis. What disease? Folate deficiency.
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Rheumatoid arthritis Tx AE?.
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Methotrexate use in rheumatoid arthritis. What disease? Folate deficiency.
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B12 deficiency physical abnormality?
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B12 deficiency abnormality? Any neurological abnormality, but 1 Peripheral neuropathy is most common, 2 Dementia is least common, 3 Postereior column damage to position and vibratory sensation, or Subacute combined degeneration of cord. Look for Ataxia. Neurological abnormalities will improve as long as they are minor (peripheral) and of short duration.
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B12 deficiency Lx association?
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B12 deficiency Lx association? Increased MethylMalonic Acid level.
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B12 and folate deficiency abnormality?
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B12 and folate deficiency abnormality? 1 Megaloblastic anemia, 2 Increased LDH and Increased Indirect Bilirubin levels, 3 Decreased Reticulocyte count, 4 Hypercellular bone marrow, 5 Macroovalocytes, 6 Increased Homocysteine levels. B12 and folate deficiency are identical hematologically and on blood smear.
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Pernicious anemia confirmatory Lx?
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Pernicious anemia confirmatory Lx? 1 Anti-Intrinsic factor, and 2 Anti-parietal cell antibodies.
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What is needed to absorb B12?
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What is needed to absorb B12? Pancreatic enzymes are needed to absorb B12. Pancreatic enzymes needed to remove B12 from R-protein so it can bind with Intrinsic factor.
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What is complication of B12 or Folate replacement?
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What is complication of B12 or Folate replacement? Hyperkalemia from massive tisue or cellular breakdown. In Pancytopenia, rapid cell production causes Hypokalemia.
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What can hemolysis leads to?
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What can hemolysis leads to? 1 Sudden Decrease in Hematocrit, 2 Indirect Bilirubin, Increased levels of LDH, and Reticulocytes, 3 Decreased Haptoglobin level, 4 Slight increase in MCV because reticulocytes are larger than normal cells, 5 Hyperkalemia from cell breakdown, 6 Folate deficiency from Increased cell production using it up
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What is breakdown product of blood?
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What is breakdown product of blood? Bilirubin. Indirect turn into Direct.
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Chronic hemolysis association?
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Chronic hemolysis association? Bilirubin Gallstone
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Hemolytic anemia with Reticulocyte always High?
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Hemolytic anemia with Reticulocyte always High? Sickle cell disease.
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Sickle cell disease cause?
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Sickle cell disease cause? Point mutation at position 6 of Beta globin chain (Valine replaces Gluatmic acid).
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Sickle cell acute painful vasoocclusive crisis cause?
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Sickle cell acute painful vasoocclusive crisis cause? 1 Hypoxia, 2 Dehydration, 3 Infection, 4 Cold temperature.
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Sickle cell disease common manifestations?
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Sickle cell disease common manifestations? 1 African American pt with Sudden, Severe pain in chest, back, and thighs (may accompany fever). 2 Bilirubin Gallstones (Chronically elevated bilirubin levels), 3 Increased infection from autosplenectomy, particularly encapsulated organisms, 4 Osteomyelitis (Salmonella), 5 Retinopathy, 6 Stroke, 7 Enlarged heart with hyperdynamic features and a systolic murmur, 8 Lower extremity skin ulcers, 9 Avascular necrosis of femoral head.
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What is the common cause of Osteomyelitis in Sickle cell disease?
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What is the common cause of Osteomyelitis in Sickle cell disease? Salmonella.
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Increased MMA. Dx?
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Increased MMA. Dx? B12 deficiency
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Sickle cell disease. Lx?
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Sickle cell disease. Lx? Best Initial Lx - Peripheral Smear. Most Accurate Lx - Hemoglobin Electrophoresis.
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No spleen. Blood smear finding?
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No spleen. Blood smear finding? Howell-Jolly bodies.
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Blood smear. Basophilic stippling. Association?
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Blood smear. Basophilic stippling. Association? Sideroblastic anemia
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What is Howell-Jolly Bodies?
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What is Howell-Jolly Bodies? Precipitated remnants of nuclear material seen inside RBC of pt with No Spleen - in Sickle cell disease
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Blood smear. Bite cells. Dx?
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Blood smear. Bite cells. Dx? G6PD
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Blood smear. Schistocyte. meaning?
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Blood smear. Schistocyte. meaning? Fragmented RBC from Intravascular hemolysis.
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Sickle cell disease - acute Tx?
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Sickle cell disease - acute Tx? 1 Oxygen_Hydration_Analgesia, 2 Fever or WBC higher - Antibiotics (Ceftriaxone, Levofloxacin, or Moxifloxacin), 3 Folic acid replacement - chronic basis, 4 Pneumococcal vaccination (autosplenectomy), 5 Hydroxyurea prevents recurrences.
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When to use Exchange Transfusion on sickle cell disease?
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When to use Exchange Transfusion on sickle cell disease? Exchange Transfusion for Severe Vasoocclusive Crisis - 1 Acute Chest Syndrome, 2 Stroke, 3 Visual disturbance from Retinal infarction, 4 Priapism.
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Parvovirus B19. Most Accurate Lx?
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Parvovirus B19. Most Accurate Lx? PCR for DNA. Parvovirus B19 causes aplastic crisis in sickle cell disease (hemocrit drop from 34 to 22 perc in 3 days. Reticulocyte count is Low).
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Isosthenuria. Dx?
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Isosthenuria. Dx? Defect in ability to concentrate urine. Sickle cell trait. Has hematuria.
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Hereditary spherocytosis defect?
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Hereditary spherocytosis defect? Spherocyte due to Spectrin defect
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Hereditary spherocytosis Lx - Initial? Lx - Most Accurate?
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Hereditary spherocytosis Lx - Initial? 1 Low MCV, 2 Increased MCHC, 3 Coombs Negative. Lx - Most Accurate? Osmotic fragility.
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Osmotic fragility Positive. Dx?
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Osmotic fragility Positive. Dx? Hereditary spherocytosis.
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Hereditary spherocytosis. Tx?
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Hereditary spherocytosis. Tx? 1 Chronic Folic Acid replacement (supports RBC production), 2 Splenectomy (Stops hemolysis).
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Bilirubin gallstones. Dx?
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Bilirubin gallstones. Dx? Hemolysis
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Warm agglutinin. Ig type?
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Warm agglutinin. Ig type? IgG (Warm weather is Great). Warm is Autoimmune hemolysis
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Cold agglutinin. Ig type?
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Cold agglutinin. Ig type? IgM - Mycoplasma, EBV. (Cold Milk)
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Autoimmune Hemolysis causes?
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Autoimmune Hemolysis causes? Warm agglutinin hemolysis cause - 1 CLL, 2 Lymphoma, 3 SLE or RA, 4 Drugs (Penicillin, Alpha-Methyldopa, Rifampin, Phenytoin)
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Autoimmune Hemolysis Tx?
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Autoimmune Hemolysis Tx? 1 Steroids, 2 Splenectomy, Severe - IVIG, 4 Rituximab (CD20 Ab).
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Alternate Tx to diminish the need for steroids in general?
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Alternate Tx to diminish the need for steroids in general? Immunosuppressive agents - 1 Cyclophosphamide, 2 Cyclosporine, 3 Azathioprine, 4 Mycophenolate mofetil
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What is cold agglutinin disease cause?
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What is cold agglutinin disease cause? Cold agglutinins are IgM antibodies against RBC in association with 1 EBV, 2 Waldenstrom Macroglobulinemia, 3 Mycoplasma Pneumonia
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Cold agglutinin. Lx?
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Cold agglutinin. Lx? Direct Coombs test Positive only for Complement.
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Cold agglutinin. Tx?
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Cold agglutinin. Tx? 1 Stay warm, 2 Rituximab, 3 Immunosuppressive agents (stop Ab production) - Cyclophosphamide, Cyclosporine.
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Cryoglobulin vs Cold agglutinin?
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Cryoglobulin vs Cold agglutinin? both are IgM and Do not respond to steroids. Cryoglobulins are associated wtih 1 Hepatitis C, 2 Joint Pain, 3 Glomerulonephritis. Cold agglutinins are IgM antibodies against RBC in association with 1 EBV, 2 Waldenstrom Macroglobulinemia, 3 Mycoplasma Pneumonia
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What is G6PD inheritance?
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What is G6PD inheritance? X-linked Recessive.
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What is G6PD defect?
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What is G6PD defect? inability to generate glutathione reductase and protect RBC from oxidant stress. Oxidant stress - 1 Infection, 2 Dapsone, 3 Quinidine, 4 Sulfa drugs, 5 Primaquine, 6 Nitrofurantoin, 7 Fava beans.
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G6PD Lx?
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G6PD Lx? Best initial - 1 Heinz Bodies, 2 Bite cells. Most Accurate - G6PD level after waiting 1 to 2 months after an acute episode of hemolysis.
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G6PD Tx?
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G6PD Tx? Avoid Oxident stress.
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What is HUS triad? Association?
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What is HUS triad? ART - A (Autoimmune Intravascular hemolysis - Schistocytes), R (Renal failure), T (Thrombocytopenia). Association - E Coli 0157-H7
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What is TTP pentad? Association?
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What is TTP pentad? FAT RN - F (Fever), A (Autoimmune Intravascular hemolysis - Schistocytes), T (Thrombocytopenia), R (Renal failure), N (Neurological). HUS triad plus Fever and Neurological. Association - 1 Ticlopidine, 2 Clopidogrel, 3 Cyclosporine, and 4 AIDS
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HUS and TTP Tx?
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HUS and TTP Tx? No treatment. Severe cases - Plasmapheresis (Use FFP if Plasmapheresis is delayed) or Plasma Exxchange. Do Not transfuse Platelets.
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What is Decay accelerating factor? Dx?
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What is Decay accelerating factor? Dx? DAF is Complement regulatory proteins (CD 55 and CD 59). Gene for Phosphatidylinositol class A (PIG-A) is defective. Clonal stem cell defect with increased sensitivity of RBC to complement in acidosis. Leading to hemolysis and thrombosis. Dx - Paroxysmal Nocturnal Hemoglobinuria.
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Deficient CD 55 and CD 59. Dx?
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Deficient CD 55 and CD 59. Dx? They are Decay accelerating factor. Dx - PNH
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What diseases does PNH leads to?
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What diseases does PNH leads to? 1 Aplastic anemia, 2 Myelodysplasia, or 3 Acute Leukemia.
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Most common cause of death in PNH?
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Most common cause of death in PNH? Thrombosis
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PNH Lx?
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PNH Lx? 1 CD55 and CD59, 2 Flow Cytometry
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PNH Tx?
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PNH Tx? 1 Prednisone, 2 Allogeneic bone marrow transplant, 3 Eculizumab (Inactivates C5 in complement pathway and Decreases red cell destruction. Complement overactivation is mechanism of PNH. Eculizumab is a complement Inhibitor), 4 Supportive care (Folic acid replacement, and Transfusions)
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Aplastic anemia Causes?
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Aplastic anemia Causes? 1 Infection (HIV, Hepatitis, CMV, EBV), 2 Cancer, 3 Radiation, 4 Toxin (Toluene, insecticides DDT, and Benzene), 5 Drug (Sulfa, Phenytoin, Carbamazepine, Chloramphenicol, Alcohol, Chemotherapy), 6 SLE, 7 PNH, 8 B12 and folate deficiency, 9 Thyroid Inhibiting medications (PTU and Methimazole)
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Aplastic anemia Lx?
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Aplastic anemia Lx? Most Accurate - Bone Marrow Biopsy
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Aplastic Anemia Px? Lx? Tx?
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Aplastic Anemia Px? 1 Fatigue (anemia), 2 Infection (WBC), 3 Bleeding (Thrombocytopenia). Lx? Bone Marrow Biopsy. Tx? 1 Supportive (1 Transfusion - anemia, 2 Antibiotics - Infection, 3 Platel - Bleeding). True Aplastic Anemia Tx - Allogeneic bone marrow transplantation (younger than 50 yo and match donor). Older than 50 Tx - 1 Antithymocyte Globulin (ATG), and 2 Cyclosporine (inhibit T cell). 3 Tacrolimus is alterative to Cyclosporine. Aplastic anemia acts as autoimmune disorder in which T cells attack pt own marrow.
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What is Cyclosporine?
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What is Cyclosporine? Inhibit T cells.
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What is Polycythemia vera? Px? Lx? Tx?
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What is Polycythemia vera? Unregulated overproduction of all 3 cell lines, but RBC overproduction most prominent. Mutation in JAK2 protein which regulates marow production. Low Erythhropoietin. Px? Hypertension, Splenomegaly, Bleeding from engorged blood vessels, Thrombosis from hyperviscosity. Lx? 1 Hematocrit Above 60 perc. Exclude hypoxia as a cause. 2 Oxygen levels are normal. 3 Erythropoietin Low. 4 Vitamin B12 Elevated. Lx - Most accurate - 5 JAK2. Tx? 1 Phlebotomy, 2 Hydroxyurea. A small number of pt convert to AML
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Contrast Polycythemia vera vs Renal cell cancer.
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Contrast Polycythemia vera (High Hematocrit, Low Erythropoietin) vs Renal cell cancer (High Hematocrit, High Erythropoietin)
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What is Essential Thrombocytosis? Tx?
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What is Essential Thrombocytosis? Elevated Platelet above 1 million leading to both thrombosis and bleeding. Can be difficult to distinguish from Elevated platelet count as a Reaction to another stress (Infection, Cancer, or Iron deficiency). Tx? aaa Under 60yo and Asymptomatic with Platelet under 1.5 million - No Tx. bbb Older than 60 and Thromboses or Platelet above 1.5 million - Begin Tx (1 Hydroxyurea. 2 Anagrelide is used when RBC suppression from hydroxyurea. 3 Aspirin is used for Erythromelalgia - Painful red hands in ET.)
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What disease has JAK2 mutation?
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What disease has JAK2 mutation? 1 Polycythemia vera, 2 Essential Thrombocytosis
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What is Myelofibrosis? Lx? Tx?
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What is Myelofibrosis? Disease of older person with Pancytopenia associated wtih Bone Marrow showing marked fibrosis. Blood production shifts to spleen and liver, which become markedly enlarged. Lx? 1 Teardrop-shaped cells, 2 Nucleated red blood cells on Blood smear. Tx? 1 Thalidomide, 2 Lenalidomide (they are Tumor necrosis factor inhibitors that increase bone marrow production). Occasional pt under age 50 to 55, Allogeic Bone Marrow Transplantation attempted.
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Teardrop - shaped cells, Nucleated RBC. Dx?
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Teardrop - shaped cells, Nucleated RBC. Dx? Myelofibrosis.
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What is Erythromelalgia? Dx? Tx?
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What is Erythromelalgia? Painful red hands in Essential Thrombocytopenia. Tx? Aspirin.
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What are Tumor necrosis factor inhibitors med? function?
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What are Tumor necrosis factor inhibitors med? function? 1 Thalidomide, 2 Lenalidomide (they are Tumor necrosis factor inhibitors that increase bone marrow production
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What is leukemia M3? Association?
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What is leukemia M3? M3 is Acute Promyelocytic leukemia. It is AML. Association? M3 is associated with aaa Translocation between chromosomes 15 and 17, bbb DIC, ccc Auer Rods - Eosinophilic inclusions with AML.
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Auer Rods. Dx?
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Auer Rods. Dx? AML, M3
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Translocation between Chromosomes 15 and 17. Dx?
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Translocation between Chromosomes 15 and 17. Dx? Acute Promyelocytic leukemia. M3
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What is general difference between Acute and Chronic leukemia?
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What is general difference between Acute and Chronic leukemia? Acute (Blast cells in pheriphery. More than 20 perc blast cells.), Chronic (mature cells. Less than 5 perc blast cells)
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Acute leukemia. Px? Lx? Tx?
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Acute leukemia. Px? 1 Signs of Pancytopenia (Fatigue, Infection, Bleeding)even though WBC count is normal or increased. Lx? Best initial - 1 Blood smear (Blast cells). Most Accurate - 2 Flow Cytometry. 3 Myeloperoxidase is characteristic of AML. AML and ALL Tx? 1 Chemotherapy to remove blasts (Inducing remission). Prognosis is Poor - 2 BMT. Prognosis is Good - 3 More Chemotherapy. Best indication of Prognosis of Acute Leukemia - Cytogenetics (Good Cytogenetics - less chance of relapse - more chemo. Bad Cytogenetics - more chance of relapse - immediate BMT). Cytogenetics= Chemo. Add All-Trans-Retinoic Acid ATRA to M3. Add Intrthecal Chemotherapy (Methotrexate) to ALL treatment - prevents relapse of ALL in CNS.
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AML M3 Tx?
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AML M3 Tx? Add All-Trans-Retinoic Acid ATRA to M3
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ALL Tx?
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ALL Tx? Add Intrathecal Methotrexate to ALL
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What is Leukemoid reaction?
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What is Leukemoid reaction? High neutrophil count due to a Reaction to another infection or stress. It has high LAP (Leukocyte alkaline phosphatase score) - shows leukocyte functioning.
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What is LAP score?
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LAP (Leukocyte alkaline phosphatase score) - High - Leukomoid reaction. Low - CML.
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What is BCR-ABL?
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What is BCR-ABL? BCR-ABL is 9_22 translocation - Philadelphia Chromosome - seen in CML
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What is 9_22 translocation?
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What is 9_22 translocation? BCR-ABL is 9_22 translocation - Philadelphia Chromosome - seen in CML
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What is Philadelphia Chromosome Dx?
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What is Philadelphia chromosome? BCR-ABL is 9_22 translocation - Philadelphia Chromosome - seen in CML
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CML Px? Dx? Tx?
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CML Px? 1 Persistently high WBC with all Neutrophils, 2 Pruritis after show, 3 Splenomegaly. Dx? 1 Low LAP. Most Accurate - 2 BCR-ABL done by PCR. 3 Small number of blasts (Under 5perc). Tx? 1 Tyrosine Kinase Inhibitors (Imatinib, Dasatinib, Niotinib). 2 BMT can cure CML.
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What disorder has Greatest Likelihood of all myeloproliferative disorders to transform into Acute Leukemia (Blast crisis)?
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What disorder has Greatest Likelihood of all myeloproliferative disorders to transform into Acute Leukemia (Blast crisis)? CML. 20 perc of untreated CML per year has blast crisis.
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What is Leukostasis reaction? Next step?
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What is Leukostasis reaction? High WBC (225000) causing SOB, Blurry vision, Confusion, Priapism. Next step? Leukapheresis.
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5q deletion. Dx? Tx?
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5q deletion. Dx? 5q deletion is characteristic abnormality of MDS (Myelodysplastic syndrome). Pt with 5q have a Better Prognosis than do those without it. Tx - Lenalidomide decrease transfusion dependence.
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Pelger-Huet cells. Dx?
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Pelger-Huet cells. Dx? Bilobed nucleus of WBC. Most distinct lab abnormality of MDS.
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MDS. Lx? Tx?
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MDS (Myelodysplastic syndrome). Complications of infection and bleeding lead to death before leukemia occurs. Preleukemic disorder presenting in older pt over 60 with pancytopenia despite a hypercellular bone marrow. Lx? 1 CBC (anemia with Increased MCV, nucleated RBC, a small number of blasts). 2 Marrow (Hypercellular), 3 Prussian blue stain (ringed sideroblasts), 4 Severity is based on Percentage of blasts. Tx? 1 Transfusion, 2 Erythropoietin (20 perc response), 3 Lenalidomide (5q deletion - can decrease transfusion). 4 Azacitidine - decreases transfusion dependence, but does not increase survival.
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What is CLL? Px? Lx? Tx?
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What is CLL? CLL is a Clonal Proliferation of Normal Mature Appearing B Lymphocytes that function Abnormally. Over age 50. Many are Asymptomatic at presentation with Markedly elevated WBC. Px? No unique physical finding. 1 Lymphadenopathy, 2 Spleen or Liver Enlargement, 3 Infection from Poor lymphocyte function. Lx? 1 WBC Above 20k with 80-98 perc Lymphocytes. 2 Half of pt are Hypogammaglobulinemic. (Low IgG). Anemia and Thrombocytopenia can occur from marrow infiltration or autoimmune warm IgG antibodies. CLL is paradoxical. When body needs useful antibody for an infection (it does not make). On the other hand, CLL cells attack normal RBC and Platelets. Tx? Stage 0 (Elevated WBC) and Stage 1 (Lymphadenopathy) - No Tx. Stage 2 (Hepatosplenomegaly), Stage 3 (Anemia), and Stage 4 (Thrombocytopenia) - Tx - 1 Fludarabine. 2 Fludarabine and Rituximab (advanced stage 2, 3, 4 initial Tx or Symptomatic - Severe Fatigue, Painful Nodes). 3 Refractory cases - Cyclophosphamide (more efficacy, but more toxic), 4 Mild cases - Chlorambucil, 5 Severe infection - IVIG, 6 Autoimmune Thrombocytopenia or Hemolysis - Prednisone. PCP prophylaxis Indicated in CLL.
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Which is less dangerous - Thrombocytopenia and Anemia from autoimmune effect, or From marrow infiltration with CLL cells?
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Which is less dangerous - Thrombocytopenia and Anemia from autoimmune effect, or From marrow infiltration with CLL cells? Autoimmune effect is Less dangerous - Tx - Prednisone (not the same as stage 3 and 4 disease)
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What is Hairy cell leukemia from?
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What is Hairy cell leukemia from? B cell with filamentous projections on smear.
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B cell with filamentous projections on smear. Dx?
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B cell with filamentous projections on smear. Dx? Hairy cell leukemia from - B cell with filamentous projections on smear.
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Hairy cell leukemia Px? Lx? Tx?
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Hairy cell leukemia Px? HCL presents in Middle aged men with 1 Pancytopenia, 2 Massive splenomegaly, 3 Monocytopenia, 4 Inaspirable Dry tap despite hypercllularity of marrow. Lx? Most Accurate - TRAP (Tartrate Resistant Acid Phosphatase) or CD11c. Tx? Cladribine.
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Pancytopenia, Inaspirable Dry tap despite hypercllularity of marrow. Dx?
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Hairy cell leukemia Px? HCL presents in Middle aged men with 1 Pancytopenia, 2 Massive splenomegaly, 3 Monocytopenia, 4 Inaspirable Dry tap despite hypercllularity of marrow. Lx? Most Accurate - TRAP (Tartrate Resistant Acid Phosphatase) or CD11c. Tx? Cladribine.
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TRAP. Dx?
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Hairy cell leukemia Px? HCL presents in Middle aged men with 1 Pancytopenia, 2 Massive splenomegaly, 3 Monocytopenia, 4 Inaspirable Dry tap despite hypercllularity of marrow. Lx? Most Accurate - TRAP (Tartrate Resistant Acid Phosphatase) or CD11c. Tx? Cladribine.
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CD11c. Dx?
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Hairy cell leukemia Px? HCL presents in Middle aged men with 1 Pancytopenia, 2 Massive splenomegaly, 3 Monocytopenia, 4 Inaspirable Dry tap despite hypercllularity of marrow. Lx? Most Accurate - TRAP (Tartrate Resistant Acid Phosphatase) or CD11c. Tx? Cladribine.
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What is Richter phenomenon?
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What is Richter phenomenon? Is conversion of CLL into High Grade Lymphoma, happens in 5 perc of pts.
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What is Non-Hodgkin Lymphoma? Px? Lx? Tx?
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What is Non-Hodgkin Lymphoma? Proliferation of Lymphocytes in Lymph Nodes and Spleen. NHL presents in advanced stage in 80-90 perc. NHL (Solid) and CLL (Liquiud) are similar. Px? Painless Lymphadenopathy, B Symptoms (Fever, Weight loss, Night sweats). Lx? 1 Excisional biopsy. CBC is normal usually. High LDH is worse. 2 CT scan. 3 Bone Marrow Biopsy. Tx? aaa Stage 1a and 2a - Local Disease - Local Radiation. bbb Stage 3, 4, and any B symptom - Advanced disease - Combination Chemotherapy with CHOP and Rituximab. CHOP - C (Cyclophosphamide), H (Adriamycin, Doxorubicin, or Hydroxydaunorubicin), O (Vincristine - Oncovin), P - Prednisone. Bukitt and Immunoblastic have worse prognosis.
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What is CHOP Tx? Dx?
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Non-Hodgkin Lymphoma - Stage 3, 4, and any B symptom - Advanced disease - Combination Chemotherapy with CHOP and Rituximab. CHOP - C (Cyclophosphamide), H (Adriamycin, Doxorubicin, or Hydroxydaunorubicin), O (Vincristine - Oncovin), P - Prednisone.
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What is Mucosal Associated Lymphoid Tissue? Tx?
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What is Mucosal Associated Lymphoid Tissue? Lymphoma of Stomach in association with H Pylori. Tx - H Pylori with 1 Clarithromycin and 2 Amoxicillin
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What does Lymphocyte Predominant means for Hodgkin Disease?
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What does Lymphocyte Predominant means for Hodgkin Disease? Best Prognosis
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In Hodgkin Disease, what has best prognosis?
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In Hodgkin Disease, what has best prognosis? Lymphocyte Predominant is Best Prognosis
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In Non Hodgkin Lymphoma, what is worse prognosis?
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In Non Hodgkin Lymphoma, what is worse prognosis? Burkitt and Immunoblastic
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In Non Hodgkin Lymphoma, what do Burkitt and Immunoblastic mean?
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In Non Hodgkin Lymphoma, what is worse prognosis? Burkitt and Immunoblastic
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Burkitt. Dx?
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In Non Hodgkin Lymphoma, what is worse prognosis? Burkitt and Immunoblastic
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Reed Sternberg cells. Dx?
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Reed Sternberg cells. Dx? Hodgkin Disease - Lymphoma
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Hodgkin Disease Lx? Tx?
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Hodgkin Disease Lx? 1 Excisional Biopsy - Lymphoma with Reed Sternberg Cells. 2 CT, 3 Bone Marrow Biopsy. Tx? aaa Stage 1a and 2a - Local Radiation. bbb Stage 3, 4, any B symptoms - ABVD. A (Adriamycin, Doxorubicin), B (Bleomycin), V (VinBlastine), D (Dacarbazine). Best Prognosis - Lymphocyte Predominant.
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Contrast Hodgkin Disease and NHL (Stage of discovery, Location, Cell, Prognosis)
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Contrast Hodgkin Disease (Stage of discovery - stage 1 and 2, Centers around Cervical area, Reed Sternberg Cell, Good Prognosis - Lymphocyte Predominant) and NHL (Stage of discovery - stage 3 and 4, Location - Disseminated, No Reed Sternberg Cell, Poor Prognosis - Burkitt and Immunoblastic)
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Complication of Radiation and Chemotherapy?
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Complication of Radiation and Chemotherapy? Radiation increases 1 Solid Tumor (Breast and Lung cancer). Breast cancer screening 8 years after Radiation tx. 2 Premature CAD. Chemo complication - 1 Acute Leukemia, 2 MDS, 3 NHL - 1 perc per year.
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Adriamycin AE?
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Adriamycin AE? Doxorubicin is Cardiotoxic. MUGA or Nuclear Ventriculogram is Most accurte way to check Left Ventricular Ejection fraction. MUGA scan to determine whether cardiac toxicity has occurred prior to development of symptoms.
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Doxorubicin AE?
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Doxorubicin AE? Cardiomyopathy
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Vincristine AE?
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Vincristine AE? Neuropathy
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Bleomycin AE?
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Bleomycin AE? Lung Fibrosis
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Cyclophosphamide AE?
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Cyclophosphamide AE? Hemorrhagic cystitis
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Cisplatin AE?
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Cisplatin AE? Renal and ototoxicity
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Cardiomyopathy is chemotherapy AE. Medication?
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Doxorubicin AE? Cardiomyopathy
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Neuropathy is chemotherapy AE. Medication?
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Vincristine AE? Neuropathy
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Lung Fibrosis is chemotherapy AE. Medication?
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Bleomycin AE? Lung Fibrosis
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Hemorrhagic cystitis is chemotherapy AE. Medication?
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Cyclophosphamide AE? Hemorrhagic cystitis
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Renal and ototoxicity is chemotherapy AE. Medication?
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Cisplatin AE? Renal and ototoxicity
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Contrast MM, MGUS, WM?
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Contrast Multiple Myeloma (Increase IgG, Bone Lesion, Bone Marrow Biopsy More than 10 perc Plasma cells), MGUS (Increase IgG, No Bone lesion, Less than 10 perc Plasma cells), Waldenstrom Macroglobulinemia (Increase IgM)
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MM general Px?
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MM general? CRAB - C (Hypercalcemia), R (Renal, Hyperuricemia), A (Anemia), B (Bone Pain - Lytic lesion)
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Plasma cells More than 10 perc. Dx?
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Plasma cells More than 10 perc. Dx? Multiple Myeloma - IgG and IgA
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What is MM? Px? Lx? Tx?
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What is MM? Proliferation of Plasma cells (More than 10 perc). Px? Bone Pain from Pathologic fractures. Osteoclast ACtivating factor (OAF) attacks bone. 1 Hypercalcemia. 2 Anemia. 3 Renal Failure (Bence Jones protein, Hypercalcemia and Hyperuricemia damage kidney). Lx? 1 Xray Bone - Lytic lesions, 2 Serum protein electrophoresis (SPEP) - IgG (60 perc) or IgA (25 perc) spike of a single clone - Monoclonal or M Spike. 3 Light chains or Bence-Jones protein only (Urine Immunoelectrophoresis). 4 Smear - Rouleaux (IgG paraprotein sticks to RBC - roll). 5 Most Accurate - Bone Marrow Biopsy - Greater 10 perc Plasma cells defines myeloma. Tx? 1 Dexamethasone with Lenalidomide, Bortezomib, or both. 2 Melphalan - older fragile pt that cannot tolerate adverse effect. 3 Autologous Bone Marrow Transplant with stem cell support - Under 70 yo (after Tx number 1)
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What is most common causes of death in MM?
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What is most common causes of death in MM? 1 Renal failure, 2 Infection.
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Rouleaux. Dx?
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Rouleaux. Dx? MM
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Bence Jones Protein. Dx?
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Bence Jones Protein. Dx? MM
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What is MGUS?
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What is MGUS? Monoclonal Gammopathy of Unknown Significance. 1 SPEP - IgG or IgA spikes in older pt. 2 Bone Marrow Biopsy - to exclude MM. 1 perc a year MGUS transform into myeloma. More MGUS, More Myeloma.
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What is Waldenstrom Macroglobulinemia? Px? Tx?
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What is Waldenstrom Macroglobulinemia? Malignant B cell Over produce IgM leading to Hyperviscosity. Px? 1 Engorged blood vessels in eye, 2 Mucosal bleeding. Lx? 1 SPEP - IgM spike (Hyperviscosity). Tx? 1 Plasmapheresis (remove IgM and Decrease Viscosity). 2 Long term - Chlorambucil, or Fludarabine and Prednisone (Control cells that make abnormal immunoglobulins)
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What are bleeding problem due to?
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What are bleeding problem due to? 1 Platelet problem, or 2 Factor problem. 1 Platelet problem (Superficial - Epistaxis, Gingival, Petechiae, purpura, mucosal surfaces such as gums, vaginal bleeding), or 2 Factor problem (Deep - Joints and muscles). Bleeding in Brain and GI can be either Platelet problem, or Factor problem.
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Superficial bleeding. Problem?
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Bleeding problem - 1 Platelet problem (Superficial - Epistaxis, Gingival, Petechiae, purpura, mucosal surfaces such as gums, vaginal bleeding), or 2 Factor problem (Deep - Joints and muscles). Bleeding in Brain and GI can be either Platelet problem, or Factor problem.
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Deep bleeding. Problem?
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Bleeding problem - 1 Platelet problem (Superficial - Epistaxis, Gingival, Petechiae, purpura, mucosal surfaces such as gums, vaginal bleeding), or 2 Factor problem (Deep - Joints and muscles). Bleeding in Brain and GI can be either Platelet problem, or Factor problem.
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Epistaxis, Gingival, Petechiae. Problem?
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Bleeding problem - 1 Platelet problem (Superficial - Epistaxis, Gingival, Petechiae, purpura, mucosal surfaces such as gums, vaginal bleeding), or 2 Factor problem (Deep - Joints and muscles). Bleeding in Brain and GI can be either Platelet problem, or Factor problem.
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Joints and muscles bleeding. Problem?
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Bleeding problem - 1 Platelet problem (Superficial - Epistaxis, Gingival, Petechiae, purpura, mucosal surfaces such as gums, vaginal bleeding), or 2 Factor problem (Deep - Joints and muscles). Bleeding in Brain and GI can be either Platelet problem, or Factor problem.
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ITP. Px? Lx? Tx?
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ITP. Normal Thrombocytopenia (Normal HCT, Normal WBC). Normal Spleen size. Px? Idiopathic Thrombocytopenic Purpura is Diagnosis of Exclusion. Lx? Occasional Lx - 1 Antiplatelet antibodies lack specificity, limited benefit. 2 Ultrasound or CT scan to exclude hypersplenism. 3 Megakaryocytes are elevated in number. Tx? 1 No bleeding, PLT More than 30k - No Tx, 2 Mild bleeding, PLT Less than 30k - Glucocorticoids, 3 Severe bleeding (GI and CNS), PLT Less than 10k - IVIG or Anti-Rho (Anti-D), 4 Recurrent episodes, Steroid dependent - Splenectomy, 5 Splenectomy or Steroids not effective - Rituximab or Azathioprine or Cyclosporine or Mycophenolate. 6 Splenectomy fails - Romiplostim, and Eltrombopag.
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Before splenectomy, what vaccination to give?
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Before splenectomy, what vaccination to give? 1 Neisseria Meningitidis, 2 H Influenzae, 3 Pneumococcus.
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No bleeding, PLT More than 30k. Tx?
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ITP Tx - 1 No bleeding, PLT More than 30k - No Tx, 2 Mild bleeding, PLT Less than 30k - Glucocorticoids, 3 Severe bleeding (GI and CNS), PLT Less than 10k - IVIG or Anti-Rho (Anti-D), 4 Recurrent episodes, Steroid dependent - Splenectomy, 5 Splenectomy or Steroids not effective - Rituximab or Azathioprine or Cyclosporine or Mycophenolate.
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Mild bleeding, PLT Less than 30k?
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ITP Tx - 1 No bleeding, PLT More than 30k - No Tx, 2 Mild bleeding, PLT Less than 30k - Glucocorticoids, 3 Severe bleeding (GI and CNS), PLT Less than 10k - IVIG or Anti-Rho (Anti-D), 4 Recurrent episodes, Steroid dependent - Splenectomy, 5 Splenectomy or Steroids not effective - Rituximab or Azathioprine or Cyclosporine or Mycophenolate.
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Severe bleeding (GI and CNS), PLT Less than 10k?
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ITP Tx - 1 No bleeding, PLT More than 30k - No Tx, 2 Mild bleeding, PLT Less than 30k - Glucocorticoids, 3 Severe bleeding (GI and CNS), PLT Less than 10k - IVIG or Anti-Rho (Anti-D), 4 Recurrent episodes, Steroid dependent - Splenectomy, 5 Splenectomy or Steroids not effective - Rituximab or Azathioprine or Cyclosporine or Mycophenolate.
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Recurrent episodes of bleeding, Steroid dependent?
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ITP Tx - 1 No bleeding, PLT More than 30k - No Tx, 2 Mild bleeding, PLT Less than 30k - Glucocorticoids, 3 Severe bleeding (GI and CNS), PLT Less than 10k - IVIG or Anti-Rho (Anti-D), 4 Recurrent episodes, Steroid dependent - Splenectomy, 5 Splenectomy or Steroids not effective - Rituximab or Azathioprine or Cyclosporine or Mycophenolate.
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Splenectomy or Steroids not effective for ITP bleeding?
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ITP Tx - 1 No bleeding, PLT More than 30k - No Tx, 2 Mild bleeding, PLT Less than 30k - Glucocorticoids, 3 Severe bleeding (GI and CNS), PLT Less than 10k - IVIG or Anti-Rho (Anti-D), 4 Recurrent episodes, Steroid dependent - Splenectomy, 5 Splenectomy or Steroids not effective - Rituximab or Azathioprine or Cyclosporine or Mycophenolate.
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Splenectomy not effective for ITP, Chronic Tx?
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Splenectomy not effective for ITP, Chronic Tx? 1 Romiplostim, and 2 Eltrombopag. Never for acute management.
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What is von Willebrand Disease? Px? Lx? Tx?
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What is von Willebrand Disease? Most common inherited bleeding disorder with a decrease in level or functioning of von Willebrand factor. Autosomal Dominant. Px? Platelet type of bleeding (Epistaxis, Gingival, Gums) with Normal Platelet count. VWD worsened after use of Aspirin. PTT may be elevated in half of pt. Lx? 1 Bleeding time (Increased duration of bleeding), 2 VWF (antigen) level may be Decreased, 3 Ristocetin Cofactor Assay (Detects VWF dysfunction). Tx? 1 DDAVP (Desmopressin) - releases subendothelial stores of VWF. 2 Factor 8 Replacement, or 3 VWF Concentrate.
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What is most common inherited bleeding disorder? inheritance?
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What is most common inherited bleeding disorder? Von Willebrand Disease. inheritance? Autosomal Dominant.
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Platelet type of bleeding with Normal Platelet count. Dx?
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Platelet type of bleeding with Normal Platelet count. Dx? VWD
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Ristocetin Cofactor Assay. Dx?
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Ristocetin Cofactor Assay? Detects VWF dysfunction (von Willebrand Disease)
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What are bleeding disorders?
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What are bleeding disorders? 1 Platelet type bleeding disorder - AAA ITP, BBB Von Willebrand Disease. 2 Coagulation factor disorder (too much bleeding) - (AAA Hemophilia factor 8 or 9 deficiency. BBB Factor 11 deficiency. CCC Factor 12 deficiency. DDD DIC), 3 Thrombophilia (AAA Factor 5 Leiden mutation, BBB HIT - Heparin Induced Thrombocytopenia, CCC Antiphospholipid Syndrome - 111 Lupus Anticoagulant, and 222 Anticardiolipin Antibody).
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What are platelet type bleeding disorders?
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What are platelet type bleeding disorders? Platelet type bleeding disorder - AAA ITP, BBB Von Willebrand Disease.
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What are coagulation factor disorders with bleeding problem?
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What are coagulation factor disorders with bleeding problem? 1 Too much bleeding (aaa Hemophilia factor 8 or 9 deficiency. bbb Factor 11 deficiency. ccc Factor 12 deficiency. ddd DIC)
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What are coagulation disorders with Thrombophilia?
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What are coagulation disorders with Thrombophilia? Thrombophilia (aaa Factor 5 Leiden mutation, bbb HIT - Heparin Induced Thrombocytopenia, ccc Antiphospholipid Syndrome - 111 Lupus Anticoagulant, and 222 Anticardiolipin Antibody).
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ELISA for platelet factor 4 antibodies. Dx?
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ELISA for platelet factor 4 antibodies. Dx? HIT
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Serotonin release assay. Dx?
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Serotonin release assay. Dx? HIT
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Russel Viper venom test. Dx?
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Russel Viper venom test. Dx? Lupus Anticoagulant Lx Most Accurate
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Lupus Anticoagulant Lx Most Accurate?
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Lupus Anticoagulant Lx Most Accurate? Russel Viper venom test.
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Multiple spontaneous abortion. Dx?
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Multiple spontaneous abortion. Dx? Anticardiolipin Antibody.
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PTT abnormal. Mixing study does not correct. Dx?
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PTT abnormal. Mixing study does not correct. Dx? Antiphospholipid syndromes - 1 Lupus anticoagulant, 2 Anticardiolipin Antibody.
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What is hemophilia? Lx? Tx?
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What is hemophilia? Delayed Joint or Muscle bleeding in a Male child (X-linked Recessive). Delay - due to primary hemostatic plug is with platelets. Lx? 1 PT - Normal. PTT - Prolonged. 2 Mixing Studies - with normal plasma will Correct PTT to Normal. 3 Most Accurate - Specific Assay for Factor 8 or 9. Tx - 1 DDAVP. 2 Severe bleeding with Very Low levels of factor 8 or 9 - replacement.
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What is factor 11 Deficiency? Lx? Tx?
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What is factor 11 Deficiency? Most of the time, there is No Increase in bleeding with factor 11 deficiency. With Trauma or Surgery, there is Increased Bleeding. Lx? 1 PT - Normal, PTT - Prolonged. 2 Mixing study - Correct PTT to Normal (occurs whenever there is a deficiency of clotting factors). Tx? FFP.
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What is factor 12 Deficiency? Lx? Tx?
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What is factor 11 Deficiency? No Bleeding. Lx? 1 PTT - Elevated. Tx? No Tx.
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What is Disseminated Intravascular Coagulation? Lx? Tx?
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What is Disseminated Intravascular Coagulation? DIC Does Not occur in otherwise Healthy people. Look for Risk - 1 Sepsis, 2 Burns, 3 Abruptio placenta or Amniotic Fluid Embolus, 4 Snake Bites, 5 Trauma resulting in tissue factor release, 6 Cancer. Bleeding related Both Clotting factor deficiency as well as Thrombocytopenia. Lx? 1 PT - Elevated, PTT - Elevated. 2 Platelet - Low, 3 D-Dimer and Fibrin Split Products - Elevated, 4 Fibrinogen level - Decreased (Consumed). Tx? 1 Platelets - Replace if under 50k. 2 Clotting factors - replace with FFP. 3 Cryoprecipitate - may be effective Fibrinogen replacement if FFP does not control bleeding.
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What is most common causes of thrombophilia? Tx?
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What is most common causes of thrombophilia? Most common cause of hypercoagulable states (thrombophilia) is Factor 5 Leiden mutation. Tx - Warfarin to INR of 2-3 for 6 months.
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What is HIT? Px? Lx? Tx?
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What is HIT? Heparin Induced Thrombocytopenia is more common with use of Unfractionated Heparin. Px? HIT presents 5-10 days after start of Heparin. Marked Drop of Platelet count (More than 30 perc). Venous Clots are More Common. Lx? 1 Confirm - ELISA for Platelet factor 4 (PF4) Antibodies, or 2 Serotonin Release Assay. 0 Tx? 1 Immediately Stop All Heparin-Containing products. 2 Give Direct Thrombin Inhibitors - Argatroban, Lepirudin, and Bivalirudin. 3 Warfarin after Directed Thrombin Inhibitor is Started. 4. Severe Thrombocytopenia or symptomatic - Plasmapheresis. Do Not transfuse platelets into HIT because it worsen thrombosis.
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What are Direct Thrombin Inhibitors? Tx for?
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HIT Tx - Direct Thrombin Inhibitors - Argatroban, Lepirudin, and Bivalirudin.
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What is Argatroban med class? Tx for?
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HIT Tx - Direct Thrombin Inhibitors - Argatroban, Lepirudin, and Bivalirudin.
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What is Lepirudin med class? Tx for?
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HIT Tx - Direct Thrombin Inhibitors - Argatroban, Lepirudin, and Bivalirudin.
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What is Bivalirudin med class? Tx for?
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HIT Tx - Direct Thrombin Inhibitors - Argatroban, Lepirudin, and Bivalirudin.
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What are Antiphospholipid syndromes?
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What are Antiphospholipid syndromes? 1 Lupus Anticoagulant, and 2 Anticardiolipin Antibody (asso Multiple Spontaneous Abortions). Both cause Thrombosis. Antiphospholipid syndromes - Only cause of Thrombophilia with Abnormality in PTT. Lx - Initial - 1 Mixing Study - circulating inhibitor - PTT will remain Elevated even after Mix. 2 Most Accurate Lx for Lupus Anticoagulant - Russel Viper Venom test. Tx - 1 Heparin, and 2 Warfarin (Like any cause of DVT or Pulmonary Embolus.) Antiphospholipid syndrome - may require Lifelong Anticoagulation.
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Thrombocytopena. Do not transfuse platelet. Dx?
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Thrombocytopena. Do not transfuse platelet. Dx? 1 TTP, 2 HUS, 3 HIT
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Smudge cell. Dx?
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Smudge cell. Dx? CLL
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Plasmapheresis is Tx for what Dx?
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Plasmapheresis is Tx for what Dx? 1 TTP, 2 HUS, 3 HIT, 4 Waldenstrom Macroglobulinemia, 5 Myasthenia Gravis, 6 Good Pasture syndrome, 7 Gillian Barre Syndrome
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PT and PTT elevated. Dx?
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PT and PTT elevated. Dx? DIC
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When to do Mixing study?
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When to do Mixing study? when PTT abnormal, mixing study is to see whether it corrects PTT abnormality
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When to do Bleeding Time?
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When to do Bleeding Time? When platelet count is Normal and Platelet type of bleeding (Petechia, Epistaxis, Gingival, purpura, mucosal surface such as gum, vaginal bleeding). Increase Bleeding Time means Platelet function problem. If platelet count is abnormal, Bleeding Time will be abnormal, so do not do bleeding time.
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What causes Thrombosis?
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What causes Thrombosis? 1 Polycythemia Vera, 2 PNH, 3 Sickle cell disease
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When to use Hydroxyurea?
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When to use Hydroxyurea? 1 Essential Thrombocytosis (Increase platelet), 2 sickle cell disease, 3 Polycythemia vera
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35 yo Caucasian male diagnosed with ulcerative colitis involving left and right colon eight years ago comes for routine follow up. Complains of occasional crampy abdominal pains and infrequent diarrhea. He is maintained on daily 5-aminosalicylic acid. He had an exacerbation of colitis six months ago which was treated with oral steroids and antibiotics. He smokes one pack per day. His family history is negative for inflammatory bowel disease and cancer. On physical examination, he has mild tenderness in the left lower abdominal quadrant. He asks about his risks of colon cancer and appropriate surveillance if applicable. What is the best response?
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35 yo Caucasian male diagnosed with ulcerative colitis involving left and right colon eight years ago comes for routine follow up. Complains of occasional crampy abdominal pains and infrequent diarrhea. He is maintained on daily 5-aminosalicylic acid. He had an exacerbation of colitis six months ago which was treated with oral steroids and antibiotics. He smokes one pack per day. His family history is negative for inflammatory bowel disease and cancer. On physical examination, he has mild tenderness in the left lower abdominal quadrant. He asks about his risks of colon cancer and appropriate surveillance if applicable. What is the best response? ---- Offer colonoscopy Now and then every year. Ulcerative colitis at an increased risk for development of colorectal cancer. Most guidelines state that pt should begin colonoscopy surveillance 8 years after diagnosis, possibly longer if only the left colon is involved. Once a surveillance program is started, colonoscopy should be repeated every 1-2 years to assess for development of colonic dysplasia.
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What is ulcerative colitis increased risk of developing? When to start screening?
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What is ulcerative colitis increased risk of developing? When to start screening? --- Ulcerative colitis is an increased risk for development of colorectal cancer. Most guidelines state that pt should begin colonoscopy surveillance 8 years after diagnosis, possibly longer if only the left colon is involved. Once a surveillance program is started, colonoscopy should be repeated every 1-2 years to assess for development of colonic dysplasia.
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For ulcerative colitis, what is screening plan? What involvement is less severe and longer screening period?
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For ulcerative colitis, what is screening plan? What involvement is less severe and longer screening period? --- Ulcerative colitis should begin colonoscopy surveillance 8 years after diagnosis, possibly longer if only the left colon is involved. Once a surveillance program is started, colonoscopy should be repeated every 1-2 years to assess for development of colonic dysplasia. Colonic dysplasia on biopsies is highly associated with progression to adenocarcinoma. It require a Total colectomy if dysplasia is identified.
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For ulcerative colitis, dysplasia is found on biopsy. Next Step?
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For ulcerative colitis, dysplasia is found on biopsy. Next Step? Total colectomy
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45 yo previously healthy male is brought to ER because of headaches and confusion for 2 days. Temp 100.2 F, Bp 140 over 86, Pulse 96. Mild icterus. Hemoglobin 8.4, Platelet 80k, Leukocyte 5500, BUN 30, Creatinine 2.2, Serum Calcium 10, Blood glucose 98. Peripheral blood smear shows many fragmented RBC. PT is normal. Next step?
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45 yo previously healthy male is brought to ER because of headaches and confusion for 2 days. Temp 100.2 F, Bp 140 over 86, Pulse 96. Mild icterus. Hemoglobin 8.4, Platelet 80k, Leukocyte 5500, BUN 30, Creatinine 2.2, Serum Calcium 10, Blood glucose 98. Peripheral blood smear shows many fragmented RBC. PT is normal. Next step? --- Plasma Exchange. Unexplained hemolytic anemia and Thrombocytopenia in pt with Renal Failure and Neurologic symptoms should raise strong suspicions for TTP-HUS. Fragmented cells in peripheral smear suggests Microangiopathic Hemolytic Anemia (MAHA), a characteristic finding in TTP-HUS.
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What is vom Willebrand factor cleaving protease?
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What is vom Willebrand factor cleaving protease? ADAMTS-13. Defective ADAMTS-13 is cause of von Willebrand factor multimers and platelet aggregation in TTP-HUS. Plasmapheresis repletes ADAMTS-13.
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Unexplained Hemolytic Anemia, Thrombocytopenia, Renal Failure, and Neurologic symptoms. Dx?
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Unexplained Hemolytic Anemia, Thrombocytopenia, Renal Failure, and Neurologic symptoms. Dx? TTP-HUS. ART (Anemia, Renal failure, Thrombocytopenia). FAT RN (Fever, Anemia, Thrombocytopenia, Renal failure, Neurological symptoms).
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What is transfusion indication for healthy? CAD and CHF?
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What is transfusion indication for healthy? CAD and CHF? Transfusion is indicated at Hemoglobin Less than 7-8 for Healthy. Hemoglobin Less than 10 for CAD and CHF.
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What does Cryoprecipitate contains?
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What does Cryoprecipitate contains? Fibrinogen.
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Fibrinogen replacement. Tx?
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Fibrinogen replacement. Tx? Cryoprecipitate
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55 yo woman comes with complaints of episodes of night sweats and fever, for the last 6 weeks. She has lost 20 lb and does not feel like eating anything. Her brother was recently diagnosed with polycythemia vera. Pallor and Splenomegaly. WBC 66k, Hemoglobin 8.7, Hematocrit 30 perc, Platelet 610k. Leukocyte distribution on differential smear - Promyelocyte 1 perc, Myelocyte 8 perc, Metamyelocyte 15 perc, Bands 35 perc, Segmented neutrophils 25 perc, Lymphocytes 14 perc, MOnocytes 2 perc. What is expected in this pt?
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55 yo woman comes with complaints of episodes of night sweats and fever, for the last 6 weeks. She has lost 20 lb and does not feel like eating anything. Her brother was recently diagnosed with polycythemia vera. Pallor and Splenomegaly. WBC 66k, Hemoglobin 8.7, Hematocrit 30 perc, Platelet 610k. Leukocyte distribution on differential smear - Promyelocyte 1 perc, Myelocyte 8 perc, Metamyelocyte 15 perc, Bands 35 perc, Segmented neutrophils 25 perc, Lymphocytes 14 perc, MOnocytes 2 perc. What is expected in this pt? ---- Decreased Leukocyte Alkaline Phosphatase. CML - Leukocytosis, Anemia, Increased number of Mature Granulocytic forms (Segmented Neutrophils and Band forms) on peripheral blood film. Mostly seen after age of 50 and resents with fatigue, malaise, low-grade fever, anorexia, weight loss and bone pains. Bone Marrow biopsy - Hypercellularity with Prominent Granulocytic Hyperplasia. LAP Low. Philadelphia chromosome.
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Low LAP with Leukocytosis. Dx?
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Low LAP with Leukocytosis. Dx? CML - Leukocytosis, Anemia, Increased number of Mature Granulocytic forms (Segmented Neutrophils and Band forms) on peripheral blood film. Mostly seen after age of 50 and resents with fatigue, malaise, low-grade fever, anorexia, weight loss and bone pains. Bone Marrow biopsy - Hypercellularity with Prominent Granulocytic Hyperplasia. LAP Low. Philadelphia chromosome.
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What diseases has Low LAP score?
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What diseases has Low LAP score? 1 CML, 2 Hypophosphatemia, 3 PNH.
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Increased bone marrow iron. Dx?
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Increased bone marrow iron. Dx? 1 Hemachromatosis, 2 Anemia of Chronic disease, 3 Sideroblastosis
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Absence of measurable erythropoietin in urine. Dx?
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Absence of measurable erythropoietin in urine. Dx? Polycythemia vera
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Auer Rods. Dx?
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Auer Rods. Dx? AML
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Tartrate resistant acid phosphatase. Dx?
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Tartrate resistant acid phosphatase. Dx? Hairy cell leukemia.
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