Acute severe anaemia in an elderly patient with hereditary sphaerocytosis
- 1Department of Medicine, Regionalspital, Langenthal, Switzerland
- 2Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA
- Correspondence to: Dr Alexander Imhof, Program in Infectious Diseases, Fred Hutchinson Cancer Research Center, 1100 Fairview Ave N, D3-100, Seattle, WA 98109-1024, USA;
- Received 29 April 2002
- Accepted 27 June 2002
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).
What does the bone marrow biopsy show?
What is the most likely diagnosis?
Which investigations would you perform to confirm the diagnosis?