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

Yasser Wali, Ismail Beshlawi, Naglaa Fawaz, Aisha Alkhayat, Mahmoud Zalabany, Mohamed Elshinawy, Salam Al-Kindi, Abdul Hakim A Al-Rawas, Christoph Klein

Research output: Contribution to journalArticle

4 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)245-249
Number of pages5
JournalEuropean Journal of Haematology
Volume89
Issue number3
DOIs
Publication statusPublished - Sep 2012

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Sickle Cell Anemia
Acute Chest Syndrome
Granulocyte Colony-Stimulating Factor
Neutropenia
Mutation
Neutropenia, Severe Congenital, Autosomal Recessive 3
Therapeutics

Keywords

  • Severe congenital neutropenia
  • Sickle

ASJC Scopus subject areas

  • Hematology

Cite this

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AU - Wali, Yasser

AU - Beshlawi, Ismail

AU - Fawaz, Naglaa

AU - Alkhayat, Aisha

AU - Zalabany, Mahmoud

AU - Elshinawy, Mohamed

AU - Al-Kindi, Salam

AU - Al-Rawas, Abdul Hakim A

AU - Klein, Christoph

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