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Coexistence of sickle cell disease and severe congenital neutropenia: first impressions can be deceiving
Author(s) -
Wali Yasser,
Beshlawi Ismail,
Fawaz Naglaa,
Alkhayat Aisha,
Zalabany Mahmoud,
Elshinawy Mohamed,
AlKindi Salam,
AlRawas Abdul Hakim A.,
Klein Christoph
Publication year - 2012
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2012.01827.x
Subject(s) - medicine , neutropenia , congenital neutropenia , disease , pediatrics , acute chest syndrome , immunology , sickle cell anemia , chemotherapy
We report an O mani 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 Hb SS ‐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.