A 40-year-old patient was admitted to the hospital with acute cholecystitis and a palpable spleen. A CBC was performed: Hemoglobin, 13.0 g/dL; MCV, 80 fL; Hct, 38%; MCH, 28 pg; Retic, 7%; MCHC, 36%; RPI, 3.9; Coombs' test negative; spherocytes, 2+; anisocytosis, 2+; poikilocytosis, 2+. Which of the following disorders is consistent with the laboratory profile?

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Quizplus · Accepted Answer

The laboratory profile is consistent with hereditary spherocytosis (HS). HS is an inherited disorder characterized by spherocytes, anisocytosis, and poikilocytosis on a peripheral blood smear. The RPI is typically low in HS due to the premature destruction of spherocytes. The patient's palpable spleen is also indicative of HS, as splenomegaly is a common finding in this disorder. Autoimmune hemolytic anemia (AHA) would be expected to have a positive Coombs' test, and thalassemia and hereditary elliptocytosis (HE) would not typically demonstrate spherocytes on peripheral blood smear. Hereditary pyropoikilocytosis (HPP) is a rare disorder and is typically diagnosed in childhood, so it is unlikely in this adult patient.

A 40-Year-Old Patient Was Admitted to the Hospital with Acute