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Postgrad Med J 79:244 doi:10.1136/pmj.79.930.244
  • Self assessment questions

Acute severe anaemia in an elderly patient with hereditary sphaerocytosis

  1. A Imhof1,
  2. A Kronenberg1,
  3. R B Walter2,
  4. R A Streuli1
  1. 1Department of Medicine, Regionalspital, Langenthal, Switzerland
  2. 2Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA, USA
  1. 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;
 aimhof{at}fhcrc.org
  • Received 29 April 2002
  • Accepted 27 June 2002

Answers on 246.

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).

Figure 1

Bone marrow smear (Pappenheim-staining, original magnification × 1000).

QUESTIONS

  1. What does the bone marrow biopsy show?

  2. What is the most likely diagnosis?

  3. Which investigations would you perform to confirm the diagnosis?

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