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Flashcards in Hematology - hemolysis Deck (11):
0

Patient with recurrent episodes of hemolysis, intermittent jaundice, splenomegaly, and family history of anemia – most likely diagnosis? Patient likely to develop what other pathology? Most accurate test? Treatment?

Hereditary spherocytosis

Billirubin gallstones

Osmotic fragility test

Folic acid and splenectomy (to stop hemolysis)

1

Warm/IgG hemolysis – etiologies?

#CLL or lymphoma
#SLE
#Penicillin, phenytoin, rifampin, methyldopa

2

Warm/IgG hemolysis – Diagnosed with? Describe RBCs?

Best initial treatment? Other treatments?

Coombs test

Spherocytes (spleen bites off pieces with antibodies)

Glucocorticoids; splenectomy, IVIg, steroid alternates (cyclophosphamide, cyclosporine, azathioprine, mycophenolate)

3

Patient presents with numbness or mottling of the nose, ears, fingers. Symptoms resolve upon warming up body part – diagnosis? Test? associated with what illnesses? Treatment?

Cold agglutinin disease

Cold agglutinin titer

EBV, Waldenström, mycoplasma pneumonia

Stay warm, rituximab/immunosuppressives

4

Did not give this drug for cold agglutinin disease?

Prednisone

5

African American/Mediterranean man with sudden anemia and jaundice - diagnosis? Most common precipitants?

G6PD

Dapsone, quinidine, sulfur drugs, primaquine, nitrofurantoin

6

G6PD - Best initial test? Most accurate test?

Peripheral smear for Heinz bodies and bite cells

G6PD level 1-2 months AFTER acute episode (Normal in acute episode)

7

Patient presents with episodic dark urine with the first urination of the day. Suspected diagnosis? Mechanism? Lab findings likely show? Most common cause of death?

Paroxysmal nocturnal and hemoglobinuria

Deficiency in Decay Accelerating Factor (complement regulatory proteins CD 55 and 59) - mild acidosis during sleep leads to hemolysis

Pancytopenia

Thrombosis

8

Patient with suspected paroxysmal nocturnal hemoglobinuria – most accurate test? Treatment?

Measuring level of CD 55 and 59 (decreased) with flow cytometry

#Prednisones
#Bone marrow transplant for a cure
#eculizumab - inactivates C-5 in the complement pathway

9

Thrombosis from nocturnal hemoglobinuria affects what veins most commonly?

Mesenteric and hepatic

10

Most accurate test for a plastic anemia?

Treatment if idiopathic?

Bone marrow biopsy

#Bone marrow transplant if under 50
#Antithymocyte globulin and cyclosporine/tacrolimus if over 50

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