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229 Cards in this Set
- Front
- Back
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the number remains constant and reflects a balance between RBC production and destruction
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circulating erythrocytes
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Erythropoiesis is _________ controlled and depends on adequate supplies of _________.
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hormonally
iron, amino acids and B vitamins |
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< 250 ml/min causes tissue hypoxia – if less, kidneys secrete_________.
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eryththropoeitin
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Erythropoietin release is triggered by:
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hypoxia due to decreased RBCs, decreased oxygen availability and increased tissue demand for oxygen.
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requirements for erythropoiesis
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Erythropoietin
Iron Vitamin B12 (cyancobalamin) Folic Acid (folate) Ascorbic Acid (Vitamin C) Pyridoxine (Vitamin B6) Amino acids |
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the body stores iron in:
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Hemoglobin (65%),
Liver Spleen Bone marrow |
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Circulating iron is loosely bound to the transport protein called?
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transferrin
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how long is the life span of an RBC?
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100-120 days
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Heme is degraded to a yellow pigment called ?
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bilirubin.
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The liver secretes bilirubin into the intestines as ?
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bile
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The intestines metabolize bilirubin into ?
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urobilinogen
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MC cause of anemia worldwide
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iron deficiency anemia
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Predominant age groups: toddlers, menstruating women, pregnant women, individuals > 65 yoa
Female > Male More likely in poor and underimmunized children |
iron deficiency anemia
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Dietary deficiency
Decreased absorption Increased requirements (pregnancy, lactation) blood loss - menstrual and GI Caused by bleeding unless proven otherwise Chronic NSAID use Hemoglobinuria |
iron deficiency anemia
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iron deficiency anemia pathophys
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Depletion of iron stores leads to decreased reticulocyte count and decrease in hemoglobin production.
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Iron Deficiency Anemia:Risk Factors
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Female
Frequent blood donor Pregnancy or lactation |
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Fatiguability, Malaise
Dizziness Tachycardia Heart murmur (systolic) Palpitations Tachypnea on exertion Headaches Impaired concentration Pallor (conjunctival) Koilonychia (spoon-shaped, brittle nails) Cheilosis Smooth tongue Dysphagia (esophageal webs) Pica |
iron deficiency anemia
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Measurement of all proteins available for binding mobile iron
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total iron binding capacity (TIBC)
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major iron storage protein
Normally present in serum in concentrations directly related to iron storage |
ferritin
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represents the largest quantity of iron transporting proteins
= what % total transferrin is saturated with iron – lower in iron deficiencies |
transferrin saturation (tsat)
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Low Hgb and Hct
Low MCV Low MCHC – hypochromic Low retic count Increased RDW Peripheral smear: Hypochromic microcytic RBCs Low ferritin Low serum iron Low transferrin saturation High TIBC |
iron deficiency anemia
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does a normal ferritin exclude iron deficiency anemia?
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No. ferritin is positive acute phase reactant so the level will increase whenever inflammation is present.
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iron deficiency anemia tx
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First line is ferrous sulfate PO - Consider augmenting therapy with oral ascorbic acid
Parenteral iron is an option for refractory cases, GI disease, cases where there is continued blood loss not correctable (dialysis) - Iron dextran, iron sucrose, ferumoxytol |
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Iron Deficiency Anemia: Prognosis
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Symptoms may be alleviated within the first few days of treatment, Monitor retic count and hgb to gauge response, Excellent prognosis if underlying cause is benign.
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In cases of significant anemia, coexistent ________ or _________ should be suspected.
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iron deficiency, folic acid deficiency
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Low Hgb & Hct
Low serum iron Low tsat Normal or increased ferritin levels - Positive acute phase reactant MCV normal or slightly low Usually normocytic and normochromic |
anemia of chronic dz
Tsat extremely low which leads many practitioners to diagnose as iron deficient. In contrast to iron deficient, ferritin will be normal or increased. If ferritin < 30 this should lead you to think that the patient has a concurrent iron deficiency. |
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anemia of chronic dz tx
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Purified recombinant erythropoietin IM or IV - Procrit, Epogen, Aranesp
Iron, folate, B12 supplements |
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Pathophys of B12 and Folic Acid deficiencies
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Results in abnormal bone marrow cells or megaloblasts and macrocytic RBCs.
Development of neutrophils also affected - Large cells with hypersegmentation |
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Affects 10 – 24% of individuals > 65 yoa
4.1% prevalence in women 2.1% prevalence in men |
B12 Deficiency
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Inadequate dietary intake - obtained exclusively from dietary intake of animal products
Malabsorption - Crohn’s, surgical resection Decreased production of intrinsic factor - Pernicious anemia (Autoimmune attack on gastric intrinsic factor, Atrophic gastritis ) |
B12 Deficiency
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B12 Deficiency: Risk Factors
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Elderly
Helicobacter pylori Long term antacid use Chronic alcoholism Bariatric surgery Crohn’s Disease Intestinal resection HIV infection/AIDS vegan/vegetarian |
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Fatigue
Dyspnea on exertion Palpitations Paresthesias Ataxia Decreased position sense Memory loss Irritability Dementia |
B12 Deficiency: History
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Glossitis
Anorexia Diarrhea Neurologic sx – paresthesias, difficulty with balance Neuropsychiatric sx – cognitive dysfunction, paranoid ideations, hallucinations, dementia “Megaloblastic Madness” |
B12 Deficiency: Clinical Manifestations
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Peripheral nerves are generally affected first causing paresthesias usually in the stocking glove distribution.
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B12 deficiency
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Elevated MCV
Peripheral smear - Macrocytic megaloblastic anemia, Anisocytosis, poikilocytosis, macro-ovalcytes, hypersegmented neutrophils Reduced retic count May have leukopenia and thrombocytopenia |
B12 deficiency
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B12 IM or PO
Irreversible neurologic damage can be caused if deficiency is not corrected |
B12 Deficiency: Tx
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Folic Acid Deficiency: Epidemiology
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Elderly
Pregnant Alcoholics Chronic Disease States (CKD) |
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Folic Acid Deficiency: Etiology
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Dietary deficiency (alcoholics, anorectics)
Decreased absorption Drugs (phenytoin, sulfasalazine, TMP-SMX, methotrexate) Increased requirements (pregnancy, chronic hemolytic anemias) Loss (hemodialysis) |
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CP almost identical to B12 except neuro abnormalities
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folic acid deficiency
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Elevated MCV
Peripheral smear - Macrocytic megaloblastic anemia, Anisocytosis, poikilocytosis, macro-ovalcytes, hypersegmented neutrophils Reduced retic count May have leukopenia and thrombocytopenia methylomalonic acid level normal RBC folate level low |
folic acid deficiency
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Folic Acid Deficiency: Tx
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PO folic acid, 1 mg/d
*Large doses of folic acid may produce hematologic responses in B12 deficiency but will allow neurologic damage to progress. |
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Group of disorders in which the lifespan of the RBC is reduced - Impaired erythropoesis in bone marrow, RBC destruction rate exceeds production rate
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hemolytic anemia
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Hemolytic anemia: Intrinsic
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hereditary spherocytosis, G6PD deficiency, sickle cell anemia, thalassemia
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hemolytic anemia: Extrinsic
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immune or microangiopathic hemolytic anemias
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Lab features common to all of the hemolytic anemias.
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Decreased haptoglobin
Decreased hemoglobin Increased indirect/unconjugated bilirubin Hallmark is elevated retic count in the presence of stable or falling Hgb/Hct |
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Glycoprotein produced by the liver
Responsible for binding and clearing free hemoglobin released into the plasma |
haptoglobin
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Disorder of the RBC membrane due to an abnormal amount of a specific protein.
Results in spherically shaped RBC that is less deformable than normal RBC. Hemolysis occurs due to trapped RBCs within the spleen. |
Hereditary Spherocytosis
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Autosomal dominant disease
Most common hereditary hemolytic anemia Higher incidence in northern European populations |
Hereditary Spherocytosis
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Signs and sx of anemia may or may not be present
Palpable spleen Icterus Gallstones |
Hereditary Spherocytosis
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Anemia of varying severity
Reticulocytosis (always) Microcytosis Increased MCHC Increased indirect bilirubin (-) direct Coombs test Peripheral smear - Spherocytes, Reticulocytes |
Hereditary Spherocytosis
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Coombs Test
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Indirect: screen for aby to transfused blood cells
Direct: looks at RBC to see if its coated in aby |
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Detects hemolysis by measuring the fraction of hemoglobin released from red cells at progressively more dilute salt concentrations.
Detects hemolysis in spherocytes at salt concentrations that don’t affect normal RBCs |
Osmotic fragility testing
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Hereditary Spherocytosis: Tx
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Folic acid 1 mg/d
Splenectomy |
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X-linked recessive disorder
Affects 10 – 15% African American males |
G6PD Deficiency: Epi.
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Most patients are asymptomatic.
Hemolysis triggered by: drugs, infection, diabetic ketoacidosis, exposure to fava beans may cause chronic hemolytic anemia. |
G6PD Deficiency: Clinical Manifestations
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Drugs/Chemicals To Avoid in G6PD Deficiency
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Aspirin
Dapsone Antimalarials (quinine, primaquine, dapsone) Quinidine, procainamide Sulfa drugs Nitrofurantoin Fava beans, red wine, soy products |
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Between hemolytic episodes blood appears normal.
During hemolytic episodes: Increased reticulocyte count, Increased indirect bilirubin, Peripheral smear shows “bite” or “blister” cells |
G6PD Deficiency
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G6PD Deficiency: Tx
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Avoid oxidant drugs and substances.
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Autosomal recessive disorder of Hgb synthesis leading to chronic hemolytic anemia.
In hemoglobin S, valine is substituted for glutamine in the 6th position on the beta chain: Results in sickled RBCs, These sickled RBCs have decreased lifespan |
sickle cell anemia
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Most dangerous feature of sickle cell anemia
Can be hemolytic or vascular in nature Hemolytic – due to splenic sequestration of RBCs Vascular - occlusion of sickled cells in small arteries and venules. Leads to pain, ischemia and infarction of tissue supplied. Spontaneous or provoked by infection, dehydration or hypoxia |
Sickle Cell Crises
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Sickle Cell Anemia: Epidemiology
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1/400 African Americans have homozygous sickle cell anemia
8% African Americans have sickle cell trait (carriers) |
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Children: short stature, delayed puberty
Bony abnormalities – necrosis of metacarpals and metatarsals, poorly healing ulcers over the lower tibia Hepatomegaly, jaundice, gallstones Retinal vessel obstruction CVA Tachycardia, acute chest syndrome (fever, chest pain, increasing WBC, pulmonary infiltrates) Infection |
Sickle Cell Anemia: Clinical Manifestations
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Decreased Hct
Reticulocytosis Elevated indirect bilirubin Elevated WBC Peripheral smear: Sickled RBCs, Target cells, Howell Jolly bodies |
Sickle Cell Anemia: Lab Findings
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Sickle Cell Anemia: Dx Studies
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Hemoglobin electrophoresis
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Sickle Cell Anemia: Prognosis
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Average lifespan 40 – 50 years
death due to organ failure |
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Sickle Cell Anemia: Tx
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Folic acid supplementation (1 mg/d)
Prevent crises precipitating factors (cold, dehydration) Transfusions Pneumococcal vaccine (before age 2 + booster 3-5 yrs. later) Hepatitis B and influenza vaccinations Penicillin prophylaxis (for Strep. Pneumonia) |
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Sickle Cell Anemia: Tx
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Hydroxyurea
Stem cell transplant |
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Hereditary condition that affects the synthesis of adult hemoglobin tetramer
Classified according to the polypeptide chain(s) with deficient synthesis |
Thalassemia
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is due to deficient synthesis of the b - globin chain.
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beta - thalassemia
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due to deficient synthesis of the a - globin chain.
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alpha - thalassemia
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Thalassemia: Pathophys.
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Mutant genes suppress the rate of synthesis of globulin chains.
Deficiency in one or more chains causes decreased hgb synthesis and an imbalance between alpha and non-alpha chain production. Disruption of globin balance causes the normal hgb to build up & precipitate - This damages the cell membranes, which leads to premature cell destruction |
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Most clinically severe form of thalassemia
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Thalassemia major
Deficiency of beta chain synthesis results in accumulation of alpha chain. Aggregates and forms insoluble inclusions in bone marrow erythroid precursors causing destruction of 80% of erythroblasts. |
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Primarily seen in Asians
Silent carrier: 1 gene deleted Minor/trait: 2 genes deleted Mild microcytic anemia |
alpha thalassemia
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(Hemoglobin H Dz)
3 genes deleted Chronic anemia, pallor, splenomegaly |
alpha thalassemia major
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4 genes deleted
Stillborn, abnormal accumulation of fluid in 2 or more fetal compartments (ascites, pleural effusion..) |
hydrops fetalis
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Mediterranean (Greece, Italy)
Minor – asymptomatic Intermedia - chronic hemolytic anemia, hepatosplenomegaly, bony deformities Thalassemia major (most clinically severe form) At 6 moa, severe anemia - Growth failure, bony deformities, hepatosplenomegaly, jaundice |
beta thalassemia
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CBC - Low Hgb (T. Major < 7 g/dL), Low MCV (disproportionate to degree of anemia), Norm. or high RBCs
Peripheral smear - Hypochromic microcytic anemia, Target cells, acanthocytes Bone marrow - Hypercellular with erythroblastic hyperplasia Hemoglobin electrophoresis |
Thalassemia
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Thalassemia: Treatment
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Mild forms (α-Thalassemia, β-minor) - No treatment, just identification
Hemoglobin H - Folic acid, avoid iron supplements and oxidative drugs Thalassemia major - Allogenic bone marrow transplant is treatment of choice, Regular blood transfusions (Chelation therapy (deferasirox), Folic acid), Splenectomy with hypersplenism |
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what clears RBC from the body?
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spleen and macrophages
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Serves as direct indication of O2 – carrying capacity of the blood.
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hemoglobin (Hgb)
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normal ranges for hemoglobin
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Males 14 – 17.5 g/dL
Females 12.3 – 15.3 g/dL |
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Low in patients with anemia, pregnancy, hemolysis
Elevated in chronic hypoxic states, hyperlipidemia, high altitudes, polycythemia vera (PV) |
Hemoglobin (Hgb)
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the presence of free hgb in the blood plasma, as when intravascular hemolysis occurs (should be sequestered in RBC)
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Hemoglobinemia
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presence of hgb in the urine
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Hemoglobinuria
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Percentage volume of blood that is composed of erythrocytes.
aka packed cell volume Usually ~3 times the value of Hgb. |
hematocrit (Hct)
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normal range of hematocrit
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Males 42 – 50%
Females 36 – 45% |
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Normal number of RBCs in a given amount of blood
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Males 4.5 – 5.9 x 106 cells/µL
Females 4.1 – 5.1 x 106 cells/µL |
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Low in menstruating females, anemia, hemolysis and bone marrow suppression
High in chronic hypoxic states, high altitudes, PV |
red blood cells
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Cell form that precedes the mature RBC or erythrocyte
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reticulocyte count (retics)
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normal range of reticulocytes
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.5% - 2.5% of RBCs
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Estimate of average volume of RBCs
Can be measured directly or calculated. |
mean corpuscular volume (MCV)
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normal MCV range
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80 – 96 fL/cell
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when might there be a false increase in MCV?
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reticulocytosis - reticulocytes are larger in size than mature erythrocytes
hyperglycemia - diluting fluid is taken up by RBC so it looks larger in size |
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Amount of hemoglobin (weight) per RBC
Calculated: Hgb/RBC count |
Mean Corpuscular Hemoglobin (MCH)
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when is Mean Corpuscular Hemoglobin decreased? increased?
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deceased in microcytosis
increased in macrocytosis |
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Measure of the average concentration or percentage of hgb within a single RBC
Calculated: Hgb x 100/Hct |
Mean Corpuscular Hemoglobin Concentration (MCHC)
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Mean Corpuscular Hemoglobin Concentration (MCHC) normal range
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Normal range 33.4 – 35.5 g/dL
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when is MCHC Hypochromic (deficiency of Hgb causing RBC to appear pale)
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Iron deficiency anemia, overhydration, thalassemia
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Indication of variation in cell size
Anisocytosis – considerable variation in the size of cells that are usually uniform |
red cell distribution width (RDW)
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normal range of RDW
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Normal 11.5 – 14.5%
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Used to differentiate anemia from thalassemia
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RDW - increases in early stages of iron deficiency anemia
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The rate at which erythrocytes settle in plasma
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Erythrocyte Sedimentation Rate (ESR, sed rate)
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normal ESR
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Males 1 – 15 mm/hr
Females 1 – 20 mm/hr |
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Useful for monitoring the activity of inflammatory conditions
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ESR - is higher when the disease is active and falls when intensity decreases
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when is ESR increased?
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Infection
Malignancy Inflammatory diseases (RA, polymyalgia rheumatica) Severe anemias Chronic renal failure Temporal arteritis |
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when is ESR decreased?
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PV
Corticosteroids Spherocytosis Sickle cell anemia |
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indications for peripheral blood smear
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Anemia
Sickle cell disease Thrombocytopenia Lymphoma Disseminated intravascular coagulation Myeloproliferative disorders |
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Presence of irregularly shaped RBCs in the peripheral blood
ex: Schistocytes – helmet shaped, due to lysis, Sickle cells, Target cells |
poikilocytosis
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poikilocytosis: target cells are indicative of?
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THALASSEMIA
Iron deficiency Hemoglobinopathies Artifactual |
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Dense circular blue inclusions that represent nuclear remnants.
Presence suggests defective splenic function Also present in megaloblastic and hemolytic anemias, speherocytosis and Celiac disease |
Howell Jolly Bodies
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normal WBC
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4,400 – 11,300 cells/mm3
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when would you see leukocytosis?
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Acute infection, inflammation, tissue necrosis
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when would you see leukopenia?
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Bone marrow failure, chemo or radiation, overwhelming infections (sepsis)
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what are the 2 general classifications of WBCs?
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Granulocytes or phagocytes – leukocytes that engulf/digest other cells - Neutrophils, eosinophils, basophils
Lymphocytes – leukocytes involved in recognition of non self cells or substances |
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Phagocytize, kill and digest bacteria and yeast
Elevated during the allergic response and during parasitic infections Present in large numbers in the intestine and lungs, two locations where foreign proteins enter the body |
eosinophils
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when is there eosinophilia?
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Parasitic infections
Allergic reactions (asthma, seasonal allergies) Pulmonary eosinophilias (Aspergillosis) Others (sulfonamides, ACE inhibitors, CML) *Any allergic reaction to a drug |
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Move into the tissue and become mast cells
Contain histamine and heparin. Increased in parasitic and allergic reactions. Also increased in chronic inflammation and leukemias. |
basophils
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Precursors to macrophages
Leave circulation in 16 – 36 hours and enter the tissues where they mature into macrophages. Participate in the removal of foreign substances from the body Under certain conditions, are transformed into antigen presenting cells (APCs) |
monocytes
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Phagocytic cell that exists to ingest/digest foreign proteins. Serves as first line of defense against infection
Most abundant WBC Also termed segmented neutrophils (segs) or polymorphonuclear cells (PMNs, polys) |
neutrophils
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when do you see neutropenia?
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Radiation
Leukemias Various medications (chemotherapy) Overwhelming bacterial infections - Septicemia (mainly Gram negative bacteria) Vitamin B12 or folate deficiency Viral infections - CMV, EBV, Hepatitis, MMR, HIV |
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when do you see neutrophilia?
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physiologic: Newborn, Pregnancy, N/V, Strenuous exercise, Extreme temperatures, UV light
pathologic: Acute bacterial infection, Chronic bacterial, infection, Trauma, MI, Epinephrine, lithium, corticosteroids, Cigarette smoking |
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Make up 20 – 40% of WBCs
Give specificity and memory to the body’s defense against foreign invaders. |
lymphocytes
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3 subtypes of lymphocytes:
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T lymphocytes – cell mediated immunity
B lymphocytes – humoral immunity (antibody production) Natural killer cells |
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when do you seen lymphopenia?
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HIV
Radiation exposure Corticosteroids Lymphoma (Hodgkin’s) Aplastic anemia |
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when do you see lymphocytosis?
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Infectious mononucleosis
Viral infections - Rubella, varicella, mumps, CMV Pertussis TB Syphilis Lymphoma Toxoplasmosis |
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A series of reactions designed for stoppage of bleeding
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hemostasis
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During hemostasis, the three phases occur in rapid sequence?
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Vascular spasms – immediate vasoconstriction in response to injury
Platelet plug formation Coagulation (blood clotting) |
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Immediate but temporary closure of a blood vessel resulting from contraction of smooth muscle within the wall of the vessel.
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vascular spasm
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chemical released during formation of platelet plug
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Thromboxanes
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Function in clotting mechanism by forming temporary plug that helps seal breaks in blood vessels.
Produced in blood marrow Life expectancy 5 – 9 days |
Platelets/Thrombocytes
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normal range for Platelets/Thrombocytes
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150,000 – 440,000/µL
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Platelets bind to collagen exposed by blood vessel damage
Mediated by von Willebrand factor (VWF) |
platelet adhesion
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Activation occurs as platelets adhere to collagen
ADP, thromboxanes, and other chemicals are released from the platelet and stimulate other platelets to become activated and release chemicals (cascade of activation) |
platelet release reaction
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Surface receptors bind to fibrinogen, a plasma protein
Fibrinogen forms a bridge between the surface receptors of different platelets resulting in a platelet plug |
platelet aggregation
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this series of reactions:
Formation of prothrombinase Conversion of prothrombin to thrombin Conversion of soluble fibrinogen to insoluble fibrin by thrombin |
coagulation
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critical for initiation of blood clotting. VII
intrinsic, extrinsic, or common? |
extrinsic
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maintains process of coagulation. XII, XI, IX, VIII
intrinsic, extrinsic, or common? |
intrinsic
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activated by thrombin to form fibrin
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fibrinogen
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causes plasma to become a gel-like trap
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fibrin
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coagulation tests
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PT/INR
aPTT D-dimer |
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time it takes for clot formation to occur after the addition of thromboplastin an
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Prothrombin time (protime)
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ratio of patient’s PT to a control sample raised to a power assigned by International Sensitivity Index
Way of standardizing values from different parts of world |
INR
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These markers measure the extrinsic pathway
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PT/INR
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normal PT and INR
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PT 10 – 13 sec.
INR – .8 – 1.4 |
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clotting time in seconds after addition of partial thromboplastin, calcium chloride and an activator to patient’s plasma
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Activated Partial Thromboplastin Time (aPTT)
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Used to screen for deficiencies of intrinsic pathway
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Activated Partial Thromboplastin Time (aPTT)
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Neoantigen formed when thrombin initiates the transition of fibrinogen to fibrin and activates factor XIII to cross link the fibrin formed.
Used to diagnose or rule out thrombosis Commonly used in assessment of DIC Also elevated in DVT, PE, sickle cell anemia and thrombosis of malignancy |
D-Dimer
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Excess of platelets, often >800,000/µL
Associated with physiologic stress of infection. May be seen in polycythemia vera, chronic myelogenous leukemia, idiopathic myelofibrosis, chronic inflammation |
thrombocytosis
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clinical consequences of thrombocytosis
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thrombosis, hemorrhage, and microcirculatory disturbances.
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Platelet count < 150,000/µL
MC clinical consequences include mucosal and cutaneous bleeding. Intacranial bleeding is paramount concern, especially in elderly pts who are prone to falls. Associated with numerous drugs such as heparin and antineoplastics. Also common with radiation therapy. |
thrombocytopenia
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Present in diseases/conditions where platelet production is decreased or platelet destruction is increased.
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thrombocytopenia
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Yellow marrow
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fat and connective tissue
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red marrow
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hematopoietic cells as well as fat cells and connective tissue
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bone marrow bx indications
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Evaluation of unexplained anemia, thrombocytopenia, thrombocytosis, leukopenia, leukocytosis
Search for malignancy primary to marrow (myeloma, leukemia) or metastasis Evaluation of iron stores Evaluation of disseminated infection (TB, fungal dz) Bone marrow donor harvesting |
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sites for bone marrow bx
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Sternum
Tibia Posterior spinous process Posterior superior |
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complications of bone marrow bx
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Local bleeding, hematoma
Pain, bone fx, infection |
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Which blood types are dominant over which?
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A and B are dominant over O
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What is the only way Type O can be inherited?
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Type O is recessive, and can only be inherited with 2 O genes.
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Does the O gene code for an antigen?
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The O gene does not code for an antigen, it is a “silent allele”. This lack of an A or B antigen is called “O.”
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Normally, you possess antibodies directed towards the A and/or B antigens absent from your own red cells.
Typically, these antibodies are produced beginning around _________ of life. |
3-6 months
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Are subgroups of blood type A or B more commonly encountered?
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A
80% are group A1, the other 20% are A2 or other more rare subgroups |
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Persons who do not express the D antigen (d – which denotes a lack of D antigen) are termed?
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“Rh negative.”
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RBC antigen neither A or B, aby in serum anti-A, Anti-B, and Anti-A,B. Blood type?
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Type O
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RBC antigen A only, anti-B aby. Blood type?
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Group A
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RBC antigen B only, anti-A aby, blood type?
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Group B
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RBC antigen A and B, no aby, blood type?
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Group AB
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Anti-D almost always results from RBC exposure through?
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transfusion, pregnancy or transplant.
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The D antigen has greater immunogenicity than all other RBC antigens except?
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A and B.
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>80% of D-negative persons who receive blood that is D-positive will?
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develop anti-D.
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RBCs can carry weaker, non-detectable, or partial D antigens. Weak D is typically caused by? (More commonly seen in blacks than whites.) Partial D is rare, and is caused when? These individuals type as D positive and produce anti-D.
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fewer numbers of D antigens on the RBC surface.
the D antigen is molecularly incomplete. |
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If a pt is considered weak D positive, transfuse with?
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Rh neg blood
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if a donor is considered weak D positive, label unit?
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Rh positive
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Hemolytic dz of the newborn (HDN) is most commonly caused by?
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anti-D.
is often severe because the D antigen is well developed on fetal cells. |
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What is the fetal screen? Kleihaur-Betke or “Fetaldex®” ?
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Fetal Screen is a qualitative (positive or negative) test to determine if Rh-positive fetal cells are circulating in Rh-negative maternal blood.
The Kleihaur-Betke or “Fetaldex®” is a quantitative test to determine how much fetal blood is present. |
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passive form of anti-D given is called?
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Rh Immune globulin (RhIg, AKA RhoGam®)
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If an antibody is detected, its ______must be determined before providing a patient with RBCs. This can take 1 to several hours depending on the antibody or antibodies present.
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specificity
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Antibody Immunoglobulin also includes a Direct Antibody Test (DAT). This helps determine if the antibody detected is an alloantibody (non-self) or autoantibody (self).
If the DAT is_________, the antibody is most likely an alloantibody. If the DAT is_________, there is an autoantibody involved, and the presence of alloantibodies must be ruled out. |
negative
positive |
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Whole blood consists of? volume? where used?
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Consists of RBCs, Plasma, and Platelets
Volume is approximately 500 mLs Rarely used in U.S. hospitals, but used in the military. |
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Red Blood Cells are separated from a whole blood donation, or collected by apheresis are called?
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packed red blood cells (pRBCs or RBCs)
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Platelet product expressed from a whole blood donation.
"six-pack" |
randcm platelets
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One donor donates only platelets.
Donates 1 adult dose Typical source of platelets today. |
apheresis platelets
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Indications for Transfusion
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Significant bleeding due to thrombocytopenia or abnormal platelet function. GOAL: Maintain >50,000 for therapeutic purposes.
Prophylactically treat chemotherapy patients. GOAL: Maintain between 10,000 and 20,000 |
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To be considered “Fresh” frozen plasma, it must be frozen within ?? hours of collection.
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8
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indications for fresh frozen plasma transfusion
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Warfarin reversal
Vitamin K deficiency Patients with an increased INR and/or PTT 1.5 times normal DIC TTP Hereditary Angioedema Not typically used to treat factor deficiencies due to the commercial availability of factor concentrates. |
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Concentration of high molecular weight plasma proteins
Stored at -18°C for up to one year until thawed. Once thawed, expires in 4 hours. The only concentrated fibrinogen product currently available for systemic use. ABO compatibility does not matter. Can be used as fibrin glue for surgical purposes. |
cryoprecipitate
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indications for leukoreduction?
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Febrile non-hemolytic transfusion reactions
CMV transmission |
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indications for irradiation transfusions (blood or blood product exposed to radiation)? increases levels of _____ in blood products?
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Neonates (< 4 months)
Bone Marrow or HPC recipients Recipients for products from 1st degree family members HLA-matched products Leukemia and Lymphoma patients potassium levels |
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ONLY way to prevent Graft Versus Host Disease?
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irradiation
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MC cause of acute hemolytic transfusion reaction?
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ABO incompatibility
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symptoms of acute hemolytic transfusion reaction?
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Fever
Chills Renal Failure Low Back Pain Anxiety Fast Progression Hypotension Tachycardia |
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hemolytic transfusion reactions lab findings?
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Post-transfusion tube will show hemolysis.
DAT most likely positive. Dark urine with increased urine & serum bilirubin. Increased plasma hemoglobin. Decreased haptoglobin. Increased levels in renal function tests (BUN, Creatinine) |
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Most common transfusion reaction
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Febrile Non-Hemolytic Transfusion Reactions
Fever: >1°C increase, often within 1-2 hours of transfusion. Chills Headache Vomiting |
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common causes of Febrile Non-Hemolytic Transfusion Reactions?
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Recipient HLA antibody against Donor residual white blood cells.
Donor or Recipient cytokines causing immune response Any of numerous other reactions between granulocytes, lymphocytes, or platelets may cause an immune response and increased temperature. Patient’s underlying condition |
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Febrile Non-Hemolytic Transfusion Reactions lab findings
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Negative DAT
All samples are re-tested and correct. Very rarely, hemolysis observed. May culture product to rule out bacterial contamination |
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allergic transfusion reaction types?
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Uricaria (Hives) - Mild version
Anaphylactic - More serious condition |
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allergic transfusion reaction - uricaria symptoms/cause/tx?
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Symptoms: Hives/Rash, Flushing, Mild Angioedema can occur, Often seen 2-4 hours after transfusion (possibly later)
Causes - Antibody to donor plasma protein Treatment - Premedicate with antihistamine or medicate and continue with transfusion |
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allergic transfusion reaction - anaphylactic symptoms/cause/tx?
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Symptoms - Hypotension, Severe Urticaria, Bronchospasms, Systemic edema, Respiratory distress, Uncontrollable wheezing
Causes - Recipient antibody to donor plasma protein (Type I hypersensitivity) including IgA, haptoglobin, C4, and other cytokines, Donor cytokines, Allergy to anticoagulant Tx: Put patient in Trendelenberg Position (feet up) Antihistamines or corticosteroids in milder cases Epinephrine in severe cases. If the patient is known to be IgA deficient, give product from IgA deficient donors or give washed products. |
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dx for anaphylactic allergic transfusion reaction?
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Rule out everything
Check patient for IgA and C4 deficiencies. Check medications (ACE inhibitors) Usually immediate and severe |
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bacteral/septic transfusion reaction symptoms?
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Fever
Chills Hypotension Tachycardia Shock Often fatal if not caught early. |
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Bacterial/Septic Transfusion Reactions Dx and Tx?
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Diagnosis
Blood cultures Perform gram stain and culture on the product. Rule out other possible transfusion reactions. Treatment Supportive care Broad spectrum antibiotics Treat complications from possible shock Increase renal output. |
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Delayed Hemolytic Transfusion Reactions symptoms
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Fever
Decreasing hemoglobin Mild jaundice 1 to 3 days (may be up to 7 days) after transfusion. |
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Delayed HTR lab findings
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Previously negative antibody screen is now positive
DAT is positive when tested with IgG Possible mild hemolysis (extravascular hemolysis) Slow decrease in hemoglobin levels Slightly increased plasma hemoglobin. |
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delayed HTR cause and tx?
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Cause: Antibody to blood groups antigens other than ABO (Common culprits: Kidd, Rh, Duffy, M)
Treatment Identify antibody or antibodies that are causing the reaction and give blood that is negative for the corresponding antigen. Monitor vital signs hemoglobin |
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Transfusion Associated Graft Versus Host Disease symptoms?
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Erythroderma
Macropapular rash Fever Vomiting Diarrhea Pancytopenia |
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Transfusion Associated Graft Versus Host Disease cause and prognosis?
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Cause: Active donor lymphocytes engraft in recipient and mount an attack against tissue.
Prognosis: Almost 90% fatal |
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Transfusion Associated Graft Versus Host Disease prevention?
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Irradiate all cellular products to inactivate donor lymphocytes
Patients at risk for TA-GVHD include: Neonates Immunocompromised patients Leukemia and Lymphoma patients Patients receiving blood from a 1st degree family member (parent, siblings, child) |
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Due to recipient platelet antibodies that destroy their own platelets.
Will show thrombocytopenic purpura and bleeding 8-10 days after transfusion. Treatment: IV Ig, Crossmatched Platelets |
Post-Transfusion Purpura
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Often seen with patients who have been massively transfused (>100 units)
Increased serum ferritin and liver enzymes |
Iron overload
Tx with iron chelators |
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Caused by rapid transfusion of blood products.
The anticoagulant in blood products chelates calcium. Can cause Tetany and arrhythmias. |
hypocalcemia
tx: calcium |
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Only iatrogenic transfusion reaction
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Transfusion Associated Circulatory Overload
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pts at significant risk of Transfusion Associated Circulatory Overload
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Children
Elderly Patients with chronic normovolemic anemia Patients with cardiac disease Patients with thalassemia major Patients with sickle cell disease |
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MC cause of Transfusion Associated Circulatory Overload?
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transfusion of a unit at too fast a rate
The subsequent hypervolemia leads to congestive heart failure and pulmonary edema (which may or may not be reversible). |
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symptoms of Transfusion Associated Circulatory Overload?
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Dyspnea
Cough Cyanosis Chest discomfort Headache Tachycardia Systolic hypertension (greater than 50 mmHg increase) |
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therapy and prevention of Transfusion Associated Circulatory Overload?
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Stop the transfusion immediately!
If transfusion is critical, give the unit at the slowest possible rate. If this will cause the product to be transfused over more than four hours, the blood bank can divide the product and keep part in the blood bank. Place the patient in a sitting position. In patients with chronic normovolemic anemias, therapeutic phlebotomy to remove a plasma volume equal to the volume of blood to be given should be considered. |
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Appears to be related to leukocyte antibodies in the donor or recipient.
Usually characterized by chills, cough, fever, cyanosis, hypotension and increasing respiratory distress shortly after transfusion of blood volumes that do not usually produce hypervolemia. Heart failure, volume overload, sepsis and MI should be ruled out prior to diagnosing TRALI. More common in plasma products from multiparous female donors. |
Transfusion Related Acute Lung Injury
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Proliferation of malignant plasma cells in the bone marrow
Malignant cells replace the cells of the bone marrow leading to bone destruction |
multiple myeloma
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Disease of older adults
Median age 65 years Incidence is 2 – 3 times higher in blacks than whites 4 – 5 cases per 100,000 Americans 1% of all cancers 10% of hematologic malignancies |
multiple myeloma
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multiple myeloma risk factors
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Exposure to radiation or petroleum products
Previous diagnosis of gammopathy Genetic predisposition |
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Bone pain in back, ribs or hips
Pathologic fractures Lytic bone lesions on xray Anemia - Pallor, fatigue Infection - Due to failure of normal antibody production Streptococcus pneumoniae, Haemophilus influenzae Polyuria and nocturia are common Hyperviscosity syndrome Hypercalcemia - Polyuria, constipation, muscle weakness, confusion Weight loss |
multiple myeloma
The combination of bone pain and anemia must always raise suspicion for multiple myeloma! |
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Light chain immunoglobulins toxic to kidneys
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Bence Jones Proteins
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MC cause of hypercalcemia in hospitalized its? in outpatients?
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hospitalized: malignancy
outpatient: parathyroidism |
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Protein electrophoresis of serum and/or urine show M protein (SPEP, UPEP) - M-protein presents as a single narrow peak or as a dense discrete band
M spike |
multiple myeloma
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UPEP → Bence Jones proteinuria
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multiple myeloma
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3 criteria must be met for diagnosis of MM (+ 1 from Francis)
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Presence of M-protein in serum or urine (M spike)
Presence of ≥ 10% clonal bone marrow cells Presence of related organ or tissue impairment C – calcium level elevated R – renal insufficiency A – anemia B – bone lesions and abnormal ratio of lamda and kappa |
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complications of multiple myeloma
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Osteoporosis and fractures - Lytic bone lesions major cause of morbidity in MM
Renal failure Infections Hypercalcemia Hyperuricemia Anemia - Normochromic, normocytic Hyperviscosity |
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complications of multiple myeloma
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NEURO DZ
Radiculopathy Spinal cord compression due to a plasmacytoma or bone fragment Suspect this in patients with severe back pain along with weakness or paresthesias of the LE, or bladder or bowel dysfunction MEDICAL EMERGENCY |
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tx/management of multiple myeloma
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Observation for asymptomatic
Initial step is “induction” therapy - Oral dexamethasone, Biologic agents (lenalidomide, bortezomib) Autologous stem cell transplant Localized radiation for bone pain or tumor Bisphosphonates to help prevent fx - pamidronate, zoledronic acid Maintain adequate hydration and avoid immobilization Allopurinol if hyperuricemia present Recombinant erythropoetin if anemic |
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prognosis for multiple myeloma
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median survival = 4-6 years
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Hypercalcemia, anemia, impaired kidney fxn
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multiple myeloma
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