Long-standing moderate to marked splenomegaly suggests several differential diagnoses, both haematological and infectious, particularly leishmaniasis and malaria in endemic areas. Non-infectious causes may be missed in these regions, especially if pitfalls of serological testing are not considered. Careful patient evaluation is necessary to arrive at the correct diagnosis. We report a case of a young male whose hereditary spherocytosis was initially missed because of RK-39 positivity, splenomegaly and the fact that he hailed from an endemic region.
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