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 Blood, 15 June 2007, Vol. 109, No. 12, pp. 5157-5159.
Prepublished online as a Blood First Edition Paper on March 7, 2007; DOI 10.1182/blood-2007-02-065805.

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Submitted February 8, 2007
Accepted March 5, 2007

Fatal agranulocytosis after deferiprone therapy in a child with Diamond-Blackfan anemia

Jan-Inge Henter* and Jonas Karlen

Childhood Cancer Research Unit, Dept of Woman & Child Health, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden

* Corresponding author; email: jan-inge.henter{at}ki.se.

A 10-year-old girl with steroid-resistent Diamond-Blackfan anemia (DBA) developed agranulocytosis 9 weeks after initiating chelation with deferiprone 45 mg/kg daily (60% of recommended dose) in addition to her ordinary deferoxamine therapy. The blood counts, checked weekly, dropped markedly between weeks 8 and 9. She rapidly developed a septicemia and was admitted with high fever (40.9oC), WBC 0.4x109/L, ANC 0.1x109/L and platelets 114x109/L. She was administered broad spectrum antibiotics, G-CSF (10 microgram/kg daily) and corticosteroids but remained neutropenic and died 6 weeks after admission. Bone marrow examination day 23 revealed areas with low cellularity (around 30%), but also areas with infiltrates of T cells; granulopoiesis and erythropoiesis were scarce. We conclude that weekly neutrophil monitoring is not sufficient to avoid fatal agranulocytosis. We suggest that deferiprone is not prescribed to DBA patients, unless the clinical indications are particularly strong, and that the risk of agranulocytosis in thalassemia patients is considered carefully.


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