Step III - Heme 2
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| Cold induced hemolysis pts usually have what in their hx | Mycoplasma/EBV, complement test (+), does not respond to steroids/splenectomy/IV Ig, Coomb’s test (-)
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| What is complement testing measuring | C3/C4 usually; low means they are being used up in inflammatory/infectious conditions or an autoimmune process is occurring
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| What is another name for the direct Coombs test | Direct Anti-human globulin test
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| In what scenarios would you do a Coombs test | Eval hemolytic anemia of newborn, transfusion reactions, autoimm/drug induced hemolytic anemia
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| What is the Direct Coombs test looking for | Abs in the bloodstream attacking the Ags found on pt’s RBC; doesn’t tell what type of Abs just the presence of Abs
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| Whose blood sample is being tested in hemolytic disease of newborn | The baby
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| Whose Abs are being tested in hemolytic disease of newborn | Mother
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| Pt needs transfusion and donor blood is tested for ABO and Rh compatibility. Few days later pt has jaundice but no other symptoms. What happened | Besides A, B, AB, and Rh Ag on the RBC there are also other Ags (Kell, Kidd, Duffy, Lewis). This reaction is called Minor blood group incompatibility
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| Pt presents with sudden onset of severe hemolysis. Dx | G6PD defx
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| How is G6PD genetically transmitted and usually in which sex | X linked = male
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| MCC acute G6PD hemolysis along with other causes | MCC = Infection; oxidizing drugs eg sulfa, primiquine, dapsone/eating fava beans
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| What are Heinz bodies | Precipitated Hb attached to RBC membrane
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| Best initial test for G6PD | Heinz body test
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| Most accurate test for G6PD | G6PD level TWO MONTHS after hemolytic event (can be normal during event d/t hemolysis of defective RBCs leaving normal RBCs)
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| What kind of blood cells are seen in G6PD | Heinz bodies + bite cells
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| Tx for G6PD | Avoid oxidative stress; no Rx tx
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| Explain the pathophys behind G6PD deficiency | The PPP takes G6P (from glucose) and makes R5P via many diff enzymes, G6PD as one of them. In RBCs, G6PD reactions (2) reduce NADP to NADPH which then reduces GSSH to GSH (glutathione). The G6PD/NADPH/GSH team helps RBCs get rid of oxygen radicals
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| If pt has hemolysis presentation similar to G6PD but does not recall reactions to fava beans and medications, what is the dx | Pyruvate kinase defx
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| Pt has recurrent episodes of hemolytic anemia, splenomegaly, and INCR MCHC. Dx, most accurate test, tx | Hereditary spherocytosis, osmotic fragility test, splenectomy
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| Pt w/ Hereditary spherocytosis are at risk for developing what kind of gallstones | Bilirubin (makes sense since RBCs are breaking down)
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| What medication predisposes pts to TTP | Ticlopidine (anti-platelet drug for stroke pts)
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| What is the HUS triad | “ART” = intravascular Autoimm hemolytic Anemia/inc BUN:creatinine d/t Renal failure/Thrombocytopenia
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| What is the TTP pentad | “FN ART” = Fever/Neurological stuff/Autoimm hemolytic anemia/Renal failure/Thrombocytopenia
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| What is the tx for HUS/TTP | Most resolves on own; if severe then plasmapheresis
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| Pt exposed to E. coli, has high BUN:creatinine, low platelets, inc retic/LDH/indirect bili, low haptoglobin. Dx | HUS
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| What should you NOT give to pts w/ HUS | Antibx and platelets
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| What is the presentation of paroxysmal nocturnal hemoglobinuria | Recurrent episodes of dark urine in AM, pancytopenia
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| MCC death from PNH | Large vessel venous thrombosis (portal vein thrombosis)
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| PNH can transform into what two diseases | AML and aplastic anemia
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| What is the most accurate test for PNH | CD55 and CD59 aka decay accelerating factor
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| What older tests were used for dx PNH | Sugar water test and Ham’s test
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| Tx for PNH | Glucocorticoids
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| What study distinguishes DIC from HELLP | HELLP has NORMAL coagulation studies (PT, aPTT)
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| Pregnant lady, low platelets, high LFTs, fragmented RBCs, c/o weakness. Dx and tx | HELLP; deliver baby
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| Pt c/o sudden SOB w/o significant history, lung exam clear, CXR (-), blood drawn for labs is described as “brown.” Dx, tx | Methemoglobinemia/methylene blue
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| What kind of exposure can cause pts to develop methemoglobinemia | Nitroprusside, NO, -caine anesthetics, dapsone, amyl nitrate
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| What Ab:Ag reaction occurs in TRALI | Donor blood has Abs that attack recipient’s WBCs
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| Pt receives blood transfusion and immediately develops anaphylaxis. Dx | IgA defx
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| What is the Ab:Ag reaction that occurs in a febrile non hemolytic transfusion | Donor WBC Ag are attacked by recipient Abs
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