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Autoimmune hemolytic anemia
9%
2/23
Disseminated intravascular coagulation
0%
0/23
Drug-induced hemolytic anemia
4%
1/23
Hereditary spherocytosis
26%
6/23
Sickle cell anemia
52%
12/23
Select Answer to see Preferred Response
The patient presents with dyspnea, fatigue, jaundice, splenomegaly, and laboratory findings suggestive of hemolytic anemia. The combination of spherocytes on peripheral blood smear, positive family history, and a negative direct Coomb test suggests hereditary spherocytosis (HS) as the most likely diagnosis. HS is an autosomal dominant hemolytic anemia due to a defect in cytoskeletal proteins ankyrin and spectrin that result in red blood cell (RBC) membrane loss. Resulting spherocytes are unable to pass through the spleen and are prematurely destroyed by splenic macrophages. HS is typically diagnosed through a positive osmotic fragility test. Positive family history of anemia is contributory as this is an autosomal dominant condition. Splenectomy is indicated as treatment for moderate or severe HS. Mild HS can be treated with folate which supports erythropoiesis. Figure A shows spherocytes on peripheral blood smear. Note the lack of central pallor. Incorrect Answers: Answer 1: Autoimmune hemolytic anemia and HS present similarly (anemia, hemolysis, an elevated LDH, and a low haptoglobin). While both present with spherocytes on peripheral blood smear, AIHA produces a positive direct Coombs test, and family history should be negative for a hemolytic condition. Answer 2: Disseminated intravascular coagulation (DIC) is a microangiopathic hemolytic anemia. Patients will present with an elevated PT/PTT/d-dimer and a low platelet count. Schistocytes can be seen on peripheral smear. Answer 3: Drug-induced hemolytic anemia would present with a hemolytic anemia (anemia, jaundice, low haptoglobin, and a high LDH) after exposure to a new medication. It is less likely in this patient with spherocytes on peripheral smear. Answer 5: Sickle cell anemia presents with anemia, chronic pain crises, and sickled cells during an episode on peripheral smear. Bullet Summary: Hereditary spherocytosis presents with anemia, an elevated LDH, a low haptoglobin, splenomegaly, and spherocytes on peripheral smear.
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