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209 Cards in this Set
- Front
- Back
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When splenomegaly is "massive" where can it be palpated?
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at the umbilicus
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What are some causes of "massive" splenomegaly?
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*Myeloproliferative
*Malaria *Myelofibrosis *Usually CA until proven otherwise |
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What are some "moderate" causes of splenomegaly?
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*cirrhosis
*lymphoproliferative |
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What are some "mild" causes of splenomegaly?
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*Inflammatory (sarcoid, SLE, RA)
*Infection (hepatitis, EBV, bacterial endocarditis) *Hemotological (ITP/spherocytosis/PAU) |
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Think of the mnemonic "CHINA." What are the major causes of splenomegaly?
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C- congestion (portal HTN)
H- hemotological I- infection N- neoplasia A- autoimmune |
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What are the 4 RBC components?
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1- cell membrane
2- Hgb 3- Fe++ 4- Enzymes |
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What is a hemolytic dz that occurs at the cell membrane?
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Hereditary spherocytosis
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What is the BEST FIRST test for hereditary spherocytosis?
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Osmotic fragility test
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What is a good SCREENING test for hereditary spherocytosis?
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Blood smear
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What are two conditions that occur at the Hgb component of the RBC?
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*thalassemia
*sickle cell disease |
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What hemotological condition occurs at the enzyme component of the RBC?
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G6PD deficiency
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If the disease is not located in the bone marrow, where is it most likely going to be found?
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In the red cell
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What does the red cell distribution width (RDW) measure?
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the degree of anisocytosis -- variation in size
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The RDW is useful in the differential diagnosis of _______ anemia.
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microcytic
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Most cases of iron deficiency have a _____ RDW, and most cases of thalassemia trait have a ______ RDW.
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* raised
* normal |
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Reticulocytes are immature red blood cells that are >___ hours old.
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48
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New red cells contain ____, which they lose after 1-2 days in the circulation. This can only be seen in a specially stained blood film as a _______.
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*RNA
*Reticulum |
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On the regular blood film, reticulocytes have a grayish tinge, which is known as _________.
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polychromasia
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What is the normal retic count?
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0.5% - 2.0%
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The _____ number is better than %.
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absolute
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What does it mean and what are some causes if the retic count is increased?
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*bone marrow is in overdrive
*bleeding *hemolysis *moving to a high elevation |
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What are some causes of a decreased retic count?
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*Fe++ def
*PA *Folate/B12 def |
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If the reticulocyte count rises after successful tx for PA, iron-def, or folic acid def anemia, what does this mean?
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that the tx is working!
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Re: sickle cell disease, the greater the increase of the retic count, the greater the _______.
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hemolysis
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In a pt w/suspected anemia, what is the first test you should order? Next?
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1- CBC, then look at MCV:
*If decreased -- ferritin *if normal -- retic count *if increased -- B12/folate |
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What is the BEST test for DX of Fe+ def anemia? What is the most accurate but rarely ever done?
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*Ferritin
*Bone marrow bx |
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In any pt >40 y/o with Fe+ deficiency anemia, it is ____ until proven otherwise. What steps do you want to do next?
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*Colon CA!!
1- rectal 2- occult blood 3- colonoscopy |
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If you consider B12 deficiency, what other levels do you want to order?
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folate
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What two deficiencies do you want to consider in the alcoholic pt?
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*B12
*folate |
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What are acute phase reactants? What are some examples?
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Compounds that are increased due to a disease process, inflammation, or infection (CRP, ESR, platelets, ferritin)
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______ is the tissue storage protein for iron, NOT a transport protein.
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ferritin
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In Fe+ def anemia, a _____ must be identified.
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cause
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Hx of weight loss, change in bowel habit, hematochezia, & an abdominal mass in addition to Fe+ def, all suggest _______.
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Colon cancer
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After doing a CBC & ferritin, what is the next BEST test to DX thalassemia?
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Hb electrophoresis/HbA2
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What is the normal HbA2?
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<3.5%
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A raised HbA2 occurs in _____ ______ trait.
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beta thalassemia
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A woman has very mild anemia, but substantial microcytosis. The serum ferritin is normal; excluding Fe+ def & there is no acute phase reaction. What is the most likely dx? What test should be ordered to dx it?
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*thalassemia trait
*HbA2 (increased) |
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With "toxic" granulation/vacuolization, think _____!
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sepsis
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____ cells are called into service in times of need (i.e. infection).
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band
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_______ neutrophils represent slowed DNA synthesis.
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Hypersegmented
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What is the normal folate level?
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5-39 nmol/l
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T or F: It is possible to be severely anemic & have a normal hematocrit & hemoglobin. Why or why not?
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TRUE-- occurs when there is rapid hemorrhage, with red cells & plasma being rapidly lost simultaneously, before the body can respond by hiking up the plasma volume.
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What are some causes of general anemia?
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*lack of RBC formation
*increased RBC destruction *blood loss *or an association w/a primary disease |
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Hypochromic-microcytic anemia is classified as MCV <____; normochromic-normocytic = MCV ___-___. Macrocytic anemia is MCV > ____.
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*MCV <80
*MCV 80-100 *MCV >100 |
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If anemia occurs gradually, the plasma volume expands to accommodate the blood loss or hemolysis, in which case the pt may notice any _____ _____.
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exertional dyspnea
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What type of murmur may develop from anemia? What may this put the pt into (what condition)?
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*systolic "flow" murmur
*High-Output HF |
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What is the most accurate test for hemoglobinopathies?
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Hgb electrophoresis
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Re: hypochromic-microcytic anemias, they are caused by an abnormality in ____ synthesis.
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heme or globin
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What is the most common cause of hypochromic-microcytic anemia?
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Fe+ def secondary to chronic blood loss or low dietary intake (mainly children or preggers)
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What are some clinical features of iron def anemia?
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*fatigue, dizziness, HA
*pallor, glossitis, angular cheilosis, koilonychia *Plummer Vinson syndrome *Pica |
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What is Plummer Vinson syndrome?
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Esophageal web secondary to iron def anemia
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What is the BEST marker of iron def anemia?
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ferritin
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T or F: A normal serum ferritin rules out iron deficiency.
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No, you can't rule it out due to acute phase reactant.
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What is the Tx for Iron def anemia?
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*Ferrous sulfate, 325 mg, TID
*Take vit C to increase absorption |
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Adequate tx of Fe+ def anemia results in an increase of ___g of Hgb in ____ weeks. What may be the cause if the pt is unresponsive to therapy?
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*1
*2 weeks *noncompliance or cont bleeding |
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What test can you order to check to make sure the ferrous sulfate tx is working?
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*retic count; should increase
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For what condition is transferrin saturation the BEST test?
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Hereditary hemochromatosis
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How long should you tx Fe+ def anemia for?
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*continue until iron stores are replenished (i.e. ferritin well into normal range)
*usually 4-6 months |
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How often should ferritin be tested in the pt with iron def anemia?
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q 6 weeks
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Band neutrophils indicate ______.
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infection/sepsis
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What two medications can cause anemia of hemodilution?
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*Glitazones
*Alpha-blockers |
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1 liter of IVF will ______ the Hgb 1 gram.
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decrease
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NIMBLE is the mnemonic for causes of Fe+ def anemia. What are they?
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N- need (increased as in preggers, child w/rapid growth, etc)
I- intake (low, malnutrition) Malabsorption Blood Loss, (GI bleeding) Excessive donation |
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What is the most dangerous cause of Fe+ def anemia?
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GI bleeding
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A decrease in the Hgb can be due to _______, especially in hypovolemic pts w/pre-existing anemia.
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hemodilution
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Anemia of chronic disease may be _______ or ______.
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*normochromic-normocytic or
*hypochromic-microcytic |
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Development of anemia of chronic disease (inflammation) occurs __-__ months after onset of chronic disease.
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1-2 months
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Chronic disease leads to decreased availability of iron for _________.
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erythropoiesis
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What is the serum ferritin level in anemia of chronic disease/inflamation?
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elevated and/or normal
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In addition to ferritin, what other level is increased in ACD?
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cytokine levels
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What is the Tx for ACD?
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*usually mild & requires no tx
*resolves when underlying dz is treated |
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The term ______ ______ is the occurrence of a disease causing gene in the homozygous state or the heterozygous occurrence of a dominant hemolytic disease causing gene.
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"hemoglobin disease"
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_______ _______ is the usually asymptomatic heterozygous occurrence of a disease causing gene.
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"hemoglobin trait"
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What test is a widely used SCREENING method for sickle cell anemia?
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The sickle cell solubility test
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What is the most important DX test for Hgb S?
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Hgb electrophoresis
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_________ can shift the O2 dissociation curve.
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Hemoglobinopathies
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______ _______ occurs when a mutation in the coding region leads to an amino acid substitution in the globin chain. Give an example.
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*Structural hemoglobinopathy
*Sickle hemoglobin is the best known of these |
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What is sickle cell anemia?
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*mutation in the beta globin gene, leading to a molecule which precipitates as long polymers at low O2 tension, forcing the red cell to assume the shape of a sickle; oxidative stress
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Is sickle cell anemia reversible or permanent?
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Initially reversible, eventually becomes permanent. Red cell may then occlude a small blood vessel, causing further ischemia, more sickling, & tissue infarction
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Re: sickle cell anemia, what are "tactoids?"
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long polymers, at low oxygen tension force red cells to become sickled; seen on smear
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How is sickle cell trait dx?
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*blood film normal
*Hgb electrophoresis: HbS & HbA both present |
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What are the features of sickle cell?
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1- hemolysis
2- occlusion of small vessels by sickled red cells |
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What is the most common complication of sickle cell disease?
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painful crisis!
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What is the management of painful crisis due to sickle cell?
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*pain relief & hydration
*hydroxyurea |
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What is hydroxyurea?
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A medication that increases HbF (does NOT sickle)
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What is the overall Tx of Sickle Cell Anemia?
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*opiates & hydration for pain
*pneumovax *retinal surveillance *hydroxyurea *transfusion for serious manif. *stem cell transplant *support, folate, iron chelation |
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Normal RBCs last 120 days, but sickled cells last ____ days.
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20
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What is the genetic defect with Thalassemia?
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deletion of alpha-globin gene on chromosome 16
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What is the disorder and features of one gene defect with thalassemia?
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*alpha thalassemia
*clinically well |
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If two genes have the genetic defect with thalassemia, what is the disorder, and what are the features?
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*alpha thalassemia
*microcytosis, mild or no anemia |
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If three genes are defected with thalassemia, what is the disorder and what are the features?
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*Hemoglobin H
*Severe disease |
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If four genes are defected with thalassemia, what is the disorder and what are the features?
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*Hydrops fetalis
*Fatal in utero |
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What is the SCREENING test & what is the TX for hereditary spherocytosis?
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*osmotic fragility test
*splenectomy |
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If there is a defect on chromosome 16, what type of thalassemia is this? Chromosome 11?
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Chromosome 16 --> alpha
Chromosome 11 --> beta |
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What is an impaired production of beta chain? What are the major & minor types?
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*beta thalassemia
*minor-- heterozygous (or trait) *major-- homozygous |
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What are the features of Beta Thalassemia TRAIT?
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*NO symptoms
*Mild microcytic anemia |
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Name 3 types of MICROCYTIC anemia.
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*Fe+ def
*Thalassemia *Hereditary spherocytosis |
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What are the S&S of Beta thalassemia MAJOR?
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*No beta chain produced (no HbA)
*SEVERE microcytic anemia occurs gradually in first yr of life *marrow expansion *Fe+ overload *growth failure & death |
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Describe the different classifications of alpha thalassemia. (i.e. one gene defect, two gene defect, etc.)
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1 gene defect- mild microcytosis
2 gene defect- mild microcytosis 3 gene defect- Hgb H disease 4 gene defect- Hgb Barts- hydrops fetalis, fatal in utero |
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What is the BEST way to obtain a DNA sample to make an antenatal dx of hemoglobinopathy?
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chorionic villus sampling; or by amniocentesis; however the first way is the BEST
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What geographic location are those with thalassemia usually from?
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Mediterranean area
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Is the gene frequency for many of the hemoglobinopathies low or high?
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High
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What is the phenomenon known as "balanced polymorphism?"
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The gene frequency for many hemoglobinopathies is so high, that there is probably some compensatory advantage to their heterozygous states, despite the deleterious nature of the homozygous condition (i.e. increased resistance to malaria may be the advantage).
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The underlying genetic defect of thalassemia results in ______.
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suppression of Hgb polypeptide chain synthesis
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What is the most common form of thalassemia?
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Beta thalassemia
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Heterozygous beta thalassemias produce mild anemia, known as thalassemia ______. What does the homozygous thalassemia result in?
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*trait
*severe hemolytic anemia, where compensatory bone marrow hyperplasia produces a characteristic overgrowth of facial & skull bones |
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What are some complications of tx of thalassemia with repeated blood transfusions?
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*hemosiderosis w/cardiomyopathy
*diabetes *skin pigmentation |
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T or F: Thalassemia trait requires NO tx.
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True
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A hypochromic-microcytic anemia is found in the thalassemia trait, which may be confused with _____ anemia.
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Fe+ def
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How can the precise dx of the type of thalassemia be made?
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by Hgb electrophoresis
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How can the complication of hemosiderosis due to blood transfusions in the tx of thalassemia, be minimized?
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by the use of continuous subcutaneous infusions of the chelating agent desferrioxamine
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What is the most common hemoglobinopathy?
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sickle cell anemia
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What is the underlying genetic defect in sickle cell disease?
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a substitution of one of the amino-acid sequences in the globin, causing an unstable hemoglobin (HbS)
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Sickle cell trait is asymptomatic other than in what type of conditions?
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low oxygen tensions such as occur at high altitude or under general anesthesia
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What is seen on the peripheral blood smear of the homozygous state of sickle cell? How is the dx made?
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*target cells, poikilocytes, & irreversibly sickled cells
*Hgb electrophoresis |
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What conditions might show target cells on peripheral smear?
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*sickle cell disease or trait
*thalassemia *liver disease *hyposplenism |
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Macrocytic anemias include anemias caused by reticulocytosis, such as...?
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*acute hemorrhage
*hemolytic anemias *megaloblastic anemias |
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Name two macrocytic diseases.
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*Pernicious anemia
*B12/Folate def |
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What are some risk factors for Pernicious Anemia (PA)?
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*gastrectomy
*elderly >60 *other autoimmune terminal ileum disease |
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What are some risk factors for folate deficiency?
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*alcoholism
*preggers *hemolysis *starvation |
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What are some causes of B12 deficiency?
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*Nutritional (i.e. vegans)
*Gastrectomy *Auto-immune gastritis *Intestinal blind loop *Jejunal diverticulum *fish tapeworm *Ileal resection *Crohn's disease |
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Where does absorption of B12 occur?
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terminal ileum
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What are some causes of intravascular hemolysis?
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*acute transfusion rxns
*prosthetic heart valve dysfxn *DIC *TTP *HUS |
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What is the end-result of hemolysis?
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Free Hgb
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What organ does free Hgb damage and what condition does it cause? Is it reversible or not?
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*the kidneys
*acute tubular necrosis (ATN) *usually reversible (mannitol, tx underlying cause) |
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What does free Hgb do to urine?
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Make it positive for blood & also the pt will have a + myoglobin
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What type of cell will be seen in hemolytic disorders?
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"helmet" cell
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What are the clinical features of intravascular hemolytic anemia?
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*S&S of anemia
*restlessness, anxiety, flushing, fever/chills, HA, tachypnea, nausea, etc. |
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Serum free haptoglobin is _______ in intravascular hemolytic anemia.
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decreased
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_____ test is positive if the anemia is antibody-mediated; negative if the anemia is secondary to shearing from prosthetic valve dysfunction.
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Coombs'
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What is the Tx of intravascular hemolytic anemia?
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*mannitol
*lasix *lots of fluids *hospitalized for management of shock & DIC *osmotic diuretic is to decrease the risk of renal damage |
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The transfusion rxn of intravascular hemolytic anemia may progress to what 3 conditions?
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*shock
*renal failure *DIC |
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What type of anemia is hereditary spherocytosis?
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hemolytic
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What happens in hereditary spherocytosis re: the membrane & cytoskeleton?
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the skeleton is fragile...loss of membrane = loss of SA = loss of deformability = increased splenic clearance
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What is the most common RBC membrane defect?
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Hereditary spherocytosis
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The severe form of hereditary spherocytosis is autosomal ________.
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dominant; family hx usually positive!
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What is the BEST method of determining bleeding/clotting problems during pre-op.
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family hx
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Pts with hereditary spherocytosis (HS) have a high incidence of ______ secondary to increased bilirubin turnover.
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gallstones
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What is seen on the peripheral smear of a pt w/HS?
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*reticulocytosis
*spherocytes |
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What does the serum analysis show on a pt with HS?
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indirect hyperbilirubinemia
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What is the tx of HS?
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Splenectomy-- decreases hemolysis
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What are the clinical features of pts with HS?
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*mild-mod anemia
*splenomegaly *cholelithiasis *jaundice may occur |
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Asplenic or pts w/splenic dysfunction are at risk of overwhelming infection, most often by _______ bacteria.
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encapsulated
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What 4 vaccines are recommended for the asplenic pt?
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*Pneumococcal
*H. influenzae type B *Meningococcal group C *Influenza |
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The influenza vaccine is recommended for asplenic pts to reduce the risk of ... ?
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secondary bacterial infections
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G6PD deficiency causes _____ stress to RBCs.
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oxidative
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What two populations is G6PD deficiency common in?
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*AA
*Mediterranean |
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What conditions, foods, & drugs can cause episodic anemia (G6PD)?
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*infections
*fava beans *chinese herbal meds *sulfa *primaquine |
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What is the most common inherited red cell enzymopathy?
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G6PD deficiency
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What type of cells will be seen in G6PD?
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"bite" cells or Heinz bodies
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What is the Tx of G6PD?
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*supportive
*avoid precipitants *counsel pt/family |
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_______ is characterized by a definite increase in red blood cell mass.
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Absolute erythrocytosis
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What are some causes of absolute erythrocytosis?
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*chronic hypoxia
*EPO secreting tumors *polycythemia vera |
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______ is a condition of too much red cells.
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Polycythemia vera
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What are the clinical features of Polycythemia vera?
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*hyperviscosity
*tinnitus *light-headedness *thrombosis *High-output HF *splenomegaly |
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What happens to EPO in polycythemia vera?
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It is nearly absent... pts w/renal tumor will have increased EPO.
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What is the primary tx of polycythemia vera? If that doesn't work?
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*repeated phlebotomies
*chemotherapy (hydroxyurea) for resistant cases |
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The complications of Polycythemia vera are _______ and include... ?
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*VASCULAR
*TIAs *strokes *MI *DVT *PE |
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What are the two types of polycythemia vera?
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1- primary-- true bone marrow disorder
2- secondary -- ie hypoxia, etc. |
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How are most pts with thrombocythemia diagnosed? What is the most common presenting symptom?
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*often by chance
*thrombotic events from the raised platelet count, i.e. heart attacks, strokes, & venous thrombosis |
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What is the dx test for thrombocythemia?
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NONE!!! Dx of exclusion of other myeloproliferative diseases
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Thrombocytopenia can be due to which 3 conditions?
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1- decreased production
2- increased destruction 3- storing too many platelets in the spleen |
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What is the most common cause of abnormal bleeding?
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thrombocytopenia
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Risk of severe hemorrhage increases if platelet count is >_____.
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20,000
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What are some causes of thrombocytopenia? *PLATELETS*
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P- platelet disorders- ITP, DIC
L- leukemia A- anemia T- trauma E- enlarged spleen L- liver disease E- ethanol T- toxins: benzene, heparin S- sepsis |
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What is the BEST screening test for DIC?
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D-dimer
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DIC will have a ______ D-dimer and ______ fibrinogen.
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*increased D-dimer
*decreased fibrinogen |
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Which type of HIT is immune-mediated?
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type II
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Describe HIT type I.
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*non-immune
*mild lowering of platelet count in first 24 hours; stays above 100 *usually benign, don't need to stop the heparin |
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Describe HIT type II.
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*immune-mediated
*VERY serious! *more common with unfractionated heparin *may have thrombosis, skin lesions, DIC, etc |
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The KEY concept of HIT is the pathogenic generation of antibodies of the Ig__ class.
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G
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What test will tell you whether or not the HIT is immune-mediated?
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HIT antibody test
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What is the hallmark of vancomycin induced thrombocytopenia?
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petechiae that DOES NOT blanch
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Elevated levels of serum tryptase occur in both ______ and ______ rxns.
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anaphylactic & anaphylactoid
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Who does ITP usually occur in? What may they initially present with? What is the prognosis?
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*children; following a viral infection
*nosebleed *good prognosis |
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What are the three steps in which ITP develops?
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1- auto-antibodies develop
2- trapping of platelets in spleen & liver 3- phagocytosis by macrophages destroys the antibody coated platelets thus causing thrombocytopenia |
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Describe chronic ITP.
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*seen in adults
*more common in women *associated w/HIV infections and SLE *can be precursor of lymphoma *prognosis usually POOR |
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ITP is dx by an abnormal _____.
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bleeding time;
PT & PTT are normal |
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What is the Tx for acute ITP? Chronic?
|
Acute --> most cases require no tx; steroids, immune globulin
Chronic --> steroids, immuneglobulin, immune suppressants, splenectomy |
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What stands for Death Is Coming?
|
DIC
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DIC occurs secondary due to which conditions?
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*meningiococcemia
*sepsis *trauma *metastatic malignancy *sx- bleeding from q orifice |
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What are the typical lab findings in DIC?
|
*Prolonged PT, PTT, & BT
*elevated D-dimer *decreased fibrinogen *decreased clotting factors |
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What is the Tx of DIC?
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*reverse the cause
*FFP to replace clotting factors *platelets to correct thrombocytopenia *Prompt anticoagulation tx w/IV heparin in pts whom thrombosis predominates |
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Causes of DIC... *CLOTS*
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C- CA
L- liver disease O- hemangiOmas T- trauma- sx, post-op S- sepsis |
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What are the MC causes of DIC?
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*CA
*sepsis *Post-op |
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What is the most common hereditary coagulation disorder?
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hemophilia A
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What is hemophilia A?
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*x-linked recessive
*factor VIII deficiency |
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Clinical features of hemophilia A.
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*positive family hx
*only affects males *bleeding occurs in soft tissues, muscles, weight-bearing joints |
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Lab findings of hemophilia A...
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*prolonged PTT (reflects fxn of the intrinsic coag pathway)
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What is the Tx of hemophilia A?
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*Factor VIII concentrates or cryoprecipitates
*Avoid platelet-inhibiting drugs such as NSAIDS *don't give IM injections |
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What 3 things do you need for a platelet plug?
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*platelets
*vWF *fibrinogen |
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What does vWF do?
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*mediates platelet adhesion to endothelium
*protein carrier for Factor VIII |
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What is the most common hereditary bleeding disorder?
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Von Willebrand's disease
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What are the S&S of Von Willebrand's disease?
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similar to platelet fxn defect:
*epistaxis *easy bruising *bleeding *menorrhagia |
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Who usually picks up on a von willebrand deficiency first?
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dentists due to increased gum bleeding
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Unmarked vWD is still a common underlying cause of _______.
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hysterectomy; test women w/menorrhagia during first few days of period due to quantitative or qualitative deficiencies of vWF.
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What is the Tx for vWD? How does it work?
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*DDAVP (desmopressin)-- temporarily increases vWF & factor VIII levels 2-3x; synthetic ADH, forces cells to produce vWF
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For pts that don't respond to first-line tx of vWD what should you give?
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vWF-containing factor VIII concentrates
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Heparin affects ___(PT/PTT) and is part of the _____ pathway. What reverses the effects of heparin?
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*PTT
*intrinsic *protamine |
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Coumadin affects ___(PT/PTT) and is part of the _____ pathway. What reverses the effects of coumadin?
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*PT
*extrinsic *vit-k |
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What are the four vitamin K-dependent factors? What does vit K act as in the final step of the synthesis of these factors & by what organ?
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*2, 7, 9, & 10
*cofactor *by the liver |
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Vitamin K-dependent factor deficiencies can occur secondary to which conditions?
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*liver failure
*malabsorption of vit K (as with biliary obstruction) *malnutrition (common in ICU pts) *use of certain drugs (i.e. coumadin) |
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What are the lab findings in vit k dependent factor deficiencies?
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*PT is prolonged
*evidence of liver disease may be present |
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What is the tx of vit k dependent factor deficiencies?
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*tx underlying cause
*10 mg of parenteral vit K -- restores production of clotting factors w/in 8-10 hrs *severe bleeding with FFP |
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What does coumadin do to the action of vitamin K?
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blocks it!!
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What does the absolute neutrophil count (ANC) predict the risk of?
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serious (fatal) infection
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An ANC <____ dramatically increases the risk for infections.
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500
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What is most common cause of a decreased ANC?
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chemotherapy
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How is the ANC calculated?
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WBC x (% neutrophils + % bands)
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