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A 22-year-old man presented with longstanding history of pallor and intermittent transfusion support since the age of 10. Family history was significant with his elder brother having longstanding history of transfusion-dependent anemia, and his parents had consanguineous marriage. Examination revealed pallor, icterus, splenomegaly, and severe gouty tophi leading to deformity of fingers of the right hand (top right) and an ulceration of a toe (bottom right).
Complete blood count showed hemoglobin, 53 g/L; hematocrit 0.17 L/L; MCV 80.2 fL, white cell count 5.9 x 109/L, and platelets 395 x 109/L. On the peripheral blood smear, there was anisocytosis, polychromasia, and numerous nucleated red blood cells. Bone marrow examination (shown on the left) revealed numerous binucleated and multinucleated erythroid precursors, typical of congenital dyserythropoietic anemia type II. Hemoglobin electrophoresis showed normal levels of HbA and HbA2 and no elevation of HbF. Blood chemistry revealed raised indirect bilirubin of 2.8 mg/dL and uric acid of 16.7 mg/dL. None of the family members including the elder brother with transfusion-dependent anemia have gout.
Although gout can be associated with hemolytic anemia, the degree of severe tophaceous gout in this patient is quite unusual.
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