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A 63 year old man with known hereditary sphaerocytosis was admitted to our hospital with a two day history of fever up to 37.9°C and chills. On clinical examination, he was icteric and had splenomegaly. His blood pressure was 130/70 mm Hg, and his heart rate 72 beats/min. Haematological analysis revealed anaemia (haemoglobin 83 g/l), a raised reticulocyte count (450 × 109/l), and thrombocytopenia (95 × 109/l). A Coombs test was negative. Other laboratory findings were: alanine aminotransferase 56 IU/l, aspartate aminotransferase 40 IU/l, bilirubin 118.7 μmol/l with conjugated bilirubin 9.9 μmol/l, lactate dehydrogenase 685 U/l, the haptoglobin concentration was below the level of detection, and the international normalised ratio was 1.3. Two days after admission the patient’s temperature rose to 39.8°C, and severe anaemia was noted with a haemoglobin of 39 g/l and a significantly decreased reticulocyte count (12 × 109/l). A bone marrow biopsy was performed. The specimen was hypercellular. Hardly any red cell precursors were seen, but there were a small number of giant cells with nuclei containing eosinophilic inclusions (fig 1).
Bone marrow smear (Pappenheim-staining, original magnification × 1000).
QUESTIONS
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What does the bone marrow biopsy show?
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What is the most likely diagnosis?
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Which investigations would you perform to confirm the diagnosis?
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