Abstract
Hereditary spherocytosis is the most common hereditary hemolytic anemia. Anemia, jaundice and splenomegaly develop in hereditary spherocytosis patients as a result of the increased hemolysis following the triggering action of non-specific infections (mostly viral). Anemia, jaundice and splenomegaly may develop in patients with brucellosis as well. Hereditary spherocytosis can be confused with brucellosis as both have the same clinical signs and symptoms. We present a patient who developed hereditary hereditary spherocytosis (folic acid deficiency with hemolytic and megaloblastic crisis) that we believe was triggered by brucellosis. A 16-year-old patient presented with headache, sweating, pallor, jaundice and splenomegaly. Laboratory tests revealed a leukocyte count of 7600/µL, Hb 11 g/dL, Hct 27.1%, platelets 215,000 µ/L, MCV 92.2 fL, RDW 18.3%, MHC 37.3 pg, MHCH 40.7 g/dL, and reticulocytes 8% (corrected reticulocytes 5%). Peripheral blood smear showed a large number of macrocytes and micro-spherocytes. There was a decrease in haptoglobin and folic acid, increase in total bilirubin and LDH and increase in osmotic fragility. Brucella tube agglutination was 1/320. The patient was started folic acid, rifampicin and doxytetracycline. The symptoms disappeared, spleen dimensions regressed and the folic acid level returned to normal with treatment.In conclusion, the symptoms and signs of hereditary spherocytosis can become prominent with a triggering nonspecific infection (viral). Brucellosis should also be considered a specific trigger of hereditary spherocytosis (in patients with anemia, splenomegaly and low folic acid), especially in areas where brucellosis is endemic
Keywords: Anemia, Brucellosis, Folic acid, Hereditary, Jaundice, Spherocytosis, Splenomegaly
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