Thrombocytopenia and GBA gene mutation in a patient with adult type 1 Gaucher disease

Platelets. 2017 Dec;28(8):829-831. doi: 10.1080/09537104.2017.1306044. Epub 2017 Jun 5.

Abstract

A 38-year-old female patient was diagnosed with anemia for 3 years. Medical examination showed slight splenomegaly (250 × 62 mm), thrombocytopenia (platelets 51 × 109/L), anemia (Hb levels 107 g/L), and β-glucocerebrosidase activity (GBA) in leukocytes was lower than normal. Microscopic findings of bone marrow smear demonstrated that Gaucher cells in bone marrow and periodic acid-Schiff staining of them were positive. Sequencing of GBA genomic and cDNA identified one novel homozygous mutation, c.484A> G (p.Met162Val). This case suggests that we should pay attention to adult Gaucher disease as a differential diagnosis for cryptogenic thrombocytopenia and one novel homozygous mutation in GBA gene was reported for the first time. The novel mutation in homozygosity is apparently associated with mild, non-neuronopathic type 1 disease which is relatively uncommon in Asian populations.

Keywords: GBA gene mutation; Gaucher cells; thrombocytopenia; type 1 Gaucher disease.

Publication types

  • Letter

MeSH terms

  • Adult
  • Female
  • Gaucher Disease / complications*
  • Gaucher Disease / pathology
  • Humans
  • Mutation
  • Thrombocytopenia / etiology*
  • Thrombocytopenia / pathology