[Genetic diagnosis of a patient with non-syndromic variants of congenital neutropenia]

Zhonghua Nei Ke Za Zhi. 2011 Nov;50(11):922-5.
[Article in Chinese]

Abstract

Objective: To explore the procedures and methods for genetic diagnosis in one non-syndromic variants of congenital neutropenia (NSVCN) patient and its pathogenic mutation.

Methods: Genomic DNA was prepared from one NSVCN patient who had progressed to chronic myelomonocytic leukemia and ELA2, HAX1, WASp and GFI1 genes were amplified and sequenced.

Results: A novel compound heterogeneous mutation consisting of two frame-shift mutations (c. 430-1insG and c. 655-9del5bp) was found in HAX1 gene.

Conclusion: A practically genetic diagnosis procedure for NSVCN has been established, and the novel HAX1 gene mutation may contribute to the etiology of NSVCN.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptor Proteins, Signal Transducing / genetics*
  • Congenital Bone Marrow Failure Syndromes
  • DNA Mutational Analysis
  • Humans
  • Male
  • Mutation
  • Neutropenia / congenital*
  • Neutropenia / diagnosis
  • Neutropenia / genetics
  • Young Adult

Substances

  • Adaptor Proteins, Signal Transducing
  • HAX1 protein, human

Supplementary concepts

  • Neutropenia, Severe Congenital, Autosomal Recessive 3