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33 Cards in this Set
- Front
- Back
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Name some causes of intravascular hemolytic anemia
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-acute transfusion reaction
-prosthetic heart valve -DIC -TTP -HUS |
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What are some clinical features of intravscular hemolytic anemia?
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-s/s of anemia
-restlessness -flushing -anxiety -fever and chills -HA -chest or lumbar pain -tachypnea -nausea |
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What is seen in severe reaction form intravascular hemolytic anemia?
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-shock
-renal failure -DIC |
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In intravascular hemolytic anemia, serum free haptoglobin is increased or decreased/
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decreased
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In intravascular hemolytic anemia, Coombs test will be + if what?
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Ab are present
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If Coomb's test is negative, what does that indicate in terms of intravascular hemolytic anemia?
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that the anemia is most likely due to prosthetic valve dysfunction
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What is the tx for intravscular hemolytic anemia?
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immediate therapy with osmotic diuretic to decrease risk of renal damage
-admit for shock and DIC |
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RBC cytoskeleton normally produces what protein?
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spectrin
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What is the main problem with spherocytosis?
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spectrin is lacking
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In spherocytosis, lack of what causes RBC to loses shap and become spherical?
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-spectrin (mainly)
-ankyrin -band 4.2 -band 3 |
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What basically happens in spherocytosis that causes problems?
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-spherocytes are less deformable so they get stuck in splenic cords and are phagocytosed by macrophages
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What is the most common RBC membrane defect?
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spherocytosis
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Spherocytosis is common in who?
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Europeans
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The severe form of spherocytosis has what kind of inheritance?
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autosomal dominant
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The severe form of spherocytosis is seen in children who present how?
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with jaundice and fatigue
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What are some 2 other clinical features of spherocytosis/
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-splenomegaly
-high incidence of gall stones 2nd to increased bilirubin turnover |
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What does peripheral smear show in spherocytosis?
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-reticulocytosis and sherocytes
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What does serum analysis reveal in spherocytosis/
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indirect hyperbilirubinemia
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What type of test is done to help confirm dx of spherocytosis?
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osmotic fragility test
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What is the main tx for spherocytosis?
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splenomegaly
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What is the tx for spherocytosis?
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splenectomy
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Why is splenectomy done in spherocytosis?
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-decreases hemolyisis
-RBC life will normalize |
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G6PD defiency does what to RBCs?
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oxidative stress
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G6PD defiency causes low level of what, which leads to what?
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-low levels of Gluthione win reduced form, which leads to increased RBC oxidative damage
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Gluthione is a major scavenger of what?
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toxic species
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What happens to RBCs in G6PD defiency?
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they become rigid and non-deformable
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Because RBCs become rigid and non-deformable in G6PD defiency, they are susceptible to what/
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stagnation and destruction by RES MF in marrow, spleen, and liver
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What are some drugs that can cause G6PD defiency?
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-sulfas
-primaquine -nitrofuatonin -dapsone |
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What food is known to cause G6PD defiency?
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Fava beans
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What is the most common inherited red cell enzymopathy?
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G6PD defiency
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G6PD defiency is passed on how?
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x linked
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What are some lab findings that make dx of G6PD defiency?
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-Bite cells
-Heinz bodies -low levels of G6PD defiency |
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What is the tx for G6PD defiency?
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-supportive
-avoid precipitants -counsel family/pts |
