title: Coexistence of sickle cell disease and severe congenital neutropenia: first impressions can be deceiving.
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.
authors: Department of Child Health, Sultan Qaboos University, Muscat, Oman.
authors: Yasser
authors: Y
authors: Wali
authors: Ismail
authors: I
authors: Beshlawi
authors: Naglaa
authors: N
authors: Fawaz
authors: Aisha
authors: A
authors: Alkhayat
authors: Mahmoud
authors: M
authors: Zalabany
authors: Mohamed
authors: M
authors: Elshinawy
authors: Salam
authors: S
authors: Al-Kindi
authors: Abdul Hakim A
authors: AH
authors: Al-Rawas
authors: Christoph
authors: C
authors: Klein
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D006801:Humans; D008297:Male; D009503:Neutropenia / Q000150:complications* / Q000151:congenital