Successful Curative Therapy With Rituximab and Allogeneic Haematopoietic Stem Cell Transplantation for MALT Lymphoma Associated With STK4-Mutated CD4+ Lymphocytopenia

Pediatr Blood Cancer. 2016 Sep;63(9):1657-9. doi: 10.1002/pbc.26048. Epub 2016 May 10.

Abstract

Idiopathic CD4+ lymphocytopenia and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) are rare diseases in children. We report the first case of a child with STK4-mutated CD4+ lymphocytopenia who developed Epstein-Barr virus associated MALT lymphoma arising in the salivary gland. The child achieved complete remission with rituximab, and her immunodeficiency was cured by haematopoietic stem cell transplantation. The child remained well 24 months post transplantation.

Keywords: CD4+ lymphocytopenia; MALT lymphoma; haematopoietic stem cell transplantation; rituximab.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Agents / therapeutic use*
  • Child, Preschool
  • Female
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Lymphoma, B-Cell, Marginal Zone / etiology
  • Lymphoma, B-Cell, Marginal Zone / pathology
  • Lymphoma, B-Cell, Marginal Zone / therapy*
  • Lymphopenia / complications*
  • Mutation*
  • Protein Serine-Threonine Kinases / genetics*
  • Rituximab / therapeutic use*
  • Transplantation, Homologous

Substances

  • Antineoplastic Agents
  • Intracellular Signaling Peptides and Proteins
  • Rituximab
  • STK4 protein, human
  • Protein Serine-Threonine Kinases