Hematology - hemolysis

Question 0

Warm/IgG hemolysis – etiologies?

Answer 0

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

Question 1

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?

Answer 1

Hereditary spherocytosis

Billirubin gallstones

Osmotic fragility test

Folic acid and splenectomy (to stop hemolysis)

Question 2

Warm/IgG hemolysis – Diagnosed with? Describe RBCs?

Best initial treatment? Other treatments?

Answer 2

Coombs test

Spherocytes (spleen bites off pieces with antibodies)

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

Question 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?

Answer 3

Cold agglutinin disease

Cold agglutinin titer

EBV, Waldenström, mycoplasma pneumonia

Stay warm, rituximab/immunosuppressives

Question 4

Did not give this drug for cold agglutinin disease?

Answer 4

Prednisone

Question 5

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

Answer 5

G6PD

Dapsone, quinidine, sulfur drugs, primaquine, nitrofurantoin

Question 6

G6PD - Best initial test? Most accurate test?

Answer 6

Peripheral smear for Heinz bodies and bite cells

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

Question 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?

Answer 7

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

Question 8

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

Answer 8

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

Question 9

Thrombosis from nocturnal hemoglobinuria affects what veins most commonly?

Answer 9

Mesenteric and hepatic

Question 10

Most accurate test for a plastic anemia?

Treatment if idiopathic?

Answer 10

Bone marrow biopsy

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