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56 Cards in this Set
- Front
- Back
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what is measured in a CBC?
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number of white, red, and platelet cells
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What is hematocrit?
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RBC count X MCV
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what is anisocytosis?
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variation in size of RBCs
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what is poikilocytosis?
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variation in RBC shape
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what is polychromatophilia?
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bluish tint, of young RBCs
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what cell are all elements of the blood formed from?
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pluripotent stem cells
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What are the two multipotent progenitors that come from a pluripotent stem cell in hemotapoiesis?
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Common myeloid stem cell
common lymphoid stem cell |
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What are the only three cell types that come from a common lymphoid stem cell?
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Plasma cell, NK, and T cell
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in bone marrow, what is the normal Fat cell to hematopoietic cells?
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1:1
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What is the normal myeloid to erythroid ratio in bone marrow?
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3:1 (granulocytes: erythroid)
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What is anemia?
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this is a reduction of oxygen carrying capacity, or low red cell mass
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what are the clinical features of anemias?
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pale, weak, malaise, easy to fatigue.
Dyspnea on mild exerton Brittle/concave nails |
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What are the three main causes of anemia?
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blood loss
impaired RBC production increased RBC breakdown |
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Why is internal bleeding a little bit better during acute blood loss?
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this allows for iron to be recaptured- so you dont become iron deficient!
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How long does it take after acute blood loss, for the bone marrow to respond?
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about 7 days to mount full response
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What are the three main features of hemolytic anemias?
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premature RBC breakdown
accumulation of RBC breakdown products (bilirubin?) Increased erythropoiesis in bone marrow |
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where do hemolytic anemias typically take place?
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these usually occur extravascular- within the mononuclear phagoctye system
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What are the lab values like for intravascular hemolysis?
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anemia
hemogloublinemia hemoglobinuria jaundice decreased serum haptoglobin (which is used to bind hemoglobin in the plasma) |
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What are the signs of Extravascular hemolysis?
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anemia
jaundice reduced plasma haptoglobin Splenomegaly (which is where most break down occurs) |
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What are the findings in bone marrow and peripheral blood with hemolytic anemias?
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increased EPO
increased normoblasts in marrow reticulocytosis elevated bilirubin hemosiderosis |
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what generally causes intrinisic hemolytic anemia?
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this is a problem with the cell well itself
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What causes hereditary spherocytosis?
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intrinisic defect in red cell membrane, it is less deformable
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What is the most common cause of autosomal dominant HS? mutation?
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75% autosomal dominant, point mutation- common in northern european people (1 in 500)
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What does the molecular defect in HS lead to in red cells?
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Deficiency of ankyrin- leads to spherical shape of RBC's
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What is the peripheral smear look like in HS?
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spheroidal red cells
reticulocytosis |
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What happens to the spleen in HS?
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Marked splenic enlargement
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What are the three clinical features of HS?
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Anemia
splenomegaly jaundice |
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What is the difference between an aplastic and hemolytic crisis?
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aplastic- caused by parvovirus
hemolytic crisis- can be caused by other things |
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How is HS diagnosed?
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family history
evidence of spherocytosis by osmotic fragility |
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What procedure can help people with HS?
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splenectomy
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What causes hemolysis in G6PD?
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A person with G6PD defect has hemolysis when their older RBC's cannot handle oxidative stress, and their membranes break down
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What is the most important enzyme derangement in G6PD?
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Hereditary deficiency of G6PD activity
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What are the two clinically significant variants of G6PD?
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G6PD-A and G6PD-D
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Why do G6PD defects exist in the gene pool?
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these exist because it was helpful in west africa against malaria
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What is the common presentation of G6PD?
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hemolysis after oxidative stress like taking some medications or infections
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What is a Heinz body?
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this is precipitation of denatured hemoglobin
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What happens 2-3 days post oxidant stress in a person with G6PD?
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you see self limited acute intravascular hemolysis of older RBC's
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What is the inheritance of G6PD like?
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X linked
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What types of things cause G6PD overload, and thus hemolysis?
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antimalarial drugs
infections like hepatitis, pneumonia, typhoid Fava beans! |
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what signals recovery from a G6PD hemolysis attack?
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reticulocytosis
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What is the defect in sickle cell disease?
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defect of hemoglobin chains, causes abnormal structure
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what globin chain is affected in sickle cell disease ? gene/mutation?
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this affects Beta Chain
a point mutation places valine in the 6th position |
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What do HbS molecules do when deoxygenated?
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when deoxygenated RBC's will become sickled,- aggregate/polymerize
and stick in everything plugging up the works |
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Is HbS shape change reversible?
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it is reversible initially, but over repeated episodes the RBC becomes damaged, and irreversible.
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What four factors affect the rate and degree of sicking in sickle cell disease ?
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amount of HbS
Hemoglobin concentration (MCHC), high HbS=worse Fall in pH length of time red cells exposed to low oxygen |
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What are the anatomic alterations in sickle cell disease? why?
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Spleen that becomes smaller with age due to mini infarcts
increased bone size, due to increased demand for erythropoesis (can have a fat head) |
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what happens to bone marrow in sickle cell disease ?
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bone marrow becomes hyperplastic, may have new bone formation
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what happens to bone in sickle cell disease ?
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bone becomes bigger, and may even grow to try and accommodate more hematopoesis
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What happens to the spleen in sickle cell disease, early/late?
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early it gets large, late it gets very small and auto infarcted
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What occurs due to vascular occulsions in sickle cell disease ?
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leg ulcers, damage to many parts of the body and pain
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What are the four features of sickle cell disease that caused problems in pts?
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severe anemia
vaso occlusive issues chronic hyperbilirubinemia increased infections from encapsulated bacteria |
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what is a pain crisis in sickle cell disease ?
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hypoxia and infarctation
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whats the most common site for pain in sickle cell disease ?
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bones- MOST COMMON, lungs, liver, brain, spleen, penis
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where does sequestration crisis occur, and in who?
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children with splenomegaly, massive sequestration of deformed RBC's
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how is sickle cell disease diagnosed?
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clinical findings and peripheral smear
confirmed by tests of sickling |
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what is the Tx for sickle cell disease ?
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use hydroxyurea
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