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Eur J Haematol. 2012 Sep;89(3):245-9. doi: 10.1111/j.1600-0609.2012.01827.x.

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

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1
Department of Child Health, Sultan Qaboos University, Muscat, Oman.

Abstract

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.

[Indexed for MEDLINE]

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