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Step III

Step III - Heme 2

QuestionAnswer
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 (-)
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
What is another name for the direct Coombs test Direct Anti-human globulin test
In what scenarios would you do a Coombs test Eval hemolytic anemia of newborn, transfusion reactions, autoimm/drug induced hemolytic anemia
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
Whose blood sample is being tested in hemolytic disease of newborn The baby
Whose Abs are being tested in hemolytic disease of newborn Mother
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
Pt presents with sudden onset of severe hemolysis. Dx G6PD defx
How is G6PD genetically transmitted and usually in which sex X linked = male
MCC acute G6PD hemolysis along with other causes MCC = Infection; oxidizing drugs eg sulfa, primiquine, dapsone/eating fava beans
What are Heinz bodies Precipitated Hb attached to RBC membrane
Best initial test for G6PD Heinz body test
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)
What kind of blood cells are seen in G6PD Heinz bodies + bite cells
Tx for G6PD Avoid oxidative stress; no Rx tx
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
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
Pt has recurrent episodes of hemolytic anemia, splenomegaly, and INCR MCHC. Dx, most accurate test, tx Hereditary spherocytosis, osmotic fragility test, splenectomy
Pt w/ Hereditary spherocytosis are at risk for developing what kind of gallstones Bilirubin (makes sense since RBCs are breaking down)
What medication predisposes pts to TTP Ticlopidine (anti-platelet drug for stroke pts)
What is the HUS triad “ART” = intravascular Autoimm hemolytic Anemia/inc BUN:creatinine d/t Renal failure/Thrombocytopenia
What is the TTP pentad “FN ART” = Fever/Neurological stuff/Autoimm hemolytic anemia/Renal failure/Thrombocytopenia
What is the tx for HUS/TTP Most resolves on own; if severe then plasmapheresis
Pt exposed to E. coli, has high BUN:creatinine, low platelets, inc retic/LDH/indirect bili, low haptoglobin. Dx HUS
What should you NOT give to pts w/ HUS Antibx and platelets
What is the presentation of paroxysmal nocturnal hemoglobinuria Recurrent episodes of dark urine in AM, pancytopenia
MCC death from PNH Large vessel venous thrombosis (portal vein thrombosis)
PNH can transform into what two diseases AML and aplastic anemia
What is the most accurate test for PNH CD55 and CD59 aka decay accelerating factor
What older tests were used for dx PNH Sugar water test and Ham’s test
Tx for PNH Glucocorticoids
What study distinguishes DIC from HELLP HELLP has NORMAL coagulation studies (PT, aPTT)
Pregnant lady, low platelets, high LFTs, fragmented RBCs, c/o weakness. Dx and tx HELLP; deliver baby
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
What kind of exposure can cause pts to develop methemoglobinemia Nitroprusside, NO, -caine anesthetics, dapsone, amyl nitrate
What Ab:Ag reaction occurs in TRALI Donor blood has Abs that attack recipient’s WBCs
Pt receives blood transfusion and immediately develops anaphylaxis. Dx IgA defx
What is the Ab:Ag reaction that occurs in a febrile non hemolytic transfusion Donor WBC Ag are attacked by recipient Abs
Created by: DrINFJ