Azathioprine

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Bone marrow aplasia in a child: case report An 8-year-old boy, who had Crohn’s disease, developed bone marrow aplasia during treatment with azathioprine [route not stated]. The boy presented with fever and neutropenia after receiving azathioprine at increasing doses from 12.5 to 50 mg/day for 3 months. Laboratory tests included the following results: WBC count 0.8 × 109/L, neutrophils 0.43 × 109/L, haemoglobin 88 g/L, platelet count 131 × 109/L, reticulocyte count 3 × 109/L, and a positive sugar water test. There was a partial absence of CD55, CD59, and fluorescent aerolysin (FLAER) on granulocytes, and of CD59 on RBCs, based on flow cytometric analysis for paroxysmal nocturnal haemoglobinuria (PNH). FLAER analysis of neutrophils revealed a 67% glycosylphosphatidylinositol (GPI)-deficient clone size. He had a 6-thioguanine nucleotide metabolite (TGN) level (on azathioprine 50 mg/day) of 327 pmol/8 × 108, and an RBC level of 6-methyl mercaptopurine ribonucleotide metabolite of 807 pmol/8 × 108 RBC. Aplasia with a cellularity of 5% was evident on bone marrow aspirate and biopsy. The boy was rehydrated, and treated with anti-infectives and steroids. Azathioprine was withdrawn, and his symptoms resolved within 3 weeks, with recovery of his blood counts. The GPI-deficient population had completely disappeared on a repeat flow cytometric testing for PNH at 6 months. Author comment: "We describe the first case of transient appearance of GPI-deficient population in a patient during azathioprine-induced marrow aplasia." Al Riyami AZ, et al. Transient appearance of GPI-deficient population in a patient with azathioprine-associated bone marrow aplasia. Annals of Hematology 91: 1659-1661, No. 10, Oct 2012. Available from: URL: http://dx.doi.org/10.1007/ 803081124 s00277-012-1449-7 - Canada

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Reactions 15 Dec 2012 No. 1432