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Coexistence of sickle cell disease and severe congenital neutropenia: first impressions can be deceiving


Correspondence Dr Ismail Beshlawi, Department of Child Health, Sultan Qaboos University Hospital, PO Box 38, PC 123, Muscat, Oman. Tel: 00968 96917 397; Fax: 00968-24141136; e-mail:


We report an Omani family in whom the propositus had a rare coexistence of sickle cell disease and severe congenital neutropenia associated with a mutation in ELANE. In contrast to his siblings with sickle cell disease, the severity of HbSS-associated complications such as painful crises and acute chest syndrome was significantly reduced. His course of the disease had markedly worsened after initiating G-CSF therapy. These clinical observations suggest that neutropenia may ameliorate inflammatory responses and thus display a modulating factor with respect to the clinical course of sickle cell disease.

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