title: Successful Curative Therapy With Rituximab and Allogeneic Haematopoietic Stem Cell Transplantation for MALT Lymphoma Associated With STK4-Mutated CD4+ Lymphocytopenia.
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.
authors.affiliation: Department of Blood and Marrow Transplant, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Su Han
authors.initials: SH
authors.lastname: Lum
authors.affiliation: Department of Blood and Marrow Transplant, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Denise
authors.initials: D
authors.lastname: Bonney
authors.affiliation: Diagnostic Paediatric Histopathology Service, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Edmund
authors.initials: E
authors.lastname: Cheesman
authors.affiliation: Department of Radiology, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Neville B
authors.initials: NB
authors.lastname: Wrignt
authors.affiliation: Department of Paediatric Allergy and Immunology, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Stephen
authors.initials: S
authors.lastname: Hughes
authors.affiliation: Department of Blood and Marrow Transplant, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Robert
authors.initials: R
authors.lastname: Wynn
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D018442:Lymphoma, B-Cell, Marginal Zone / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D008231:Lymphopenia / Q000150:complications*; D009154:Mutation; D017346:Protein Serine-Threonine Kinases / Q000235:genetics*; D000069283:Rituximab / Q000627:therapeutic use*; D014184:Transplantation, Homologous; D000099317:Hippo Kinases
chemical_list: D000970:Antineoplastic Agents; D047908:Intracellular Signaling Peptides and Proteins; D017346:Protein Serine-Threonine Kinases; D000069283:Rituximab; C095566:STK4 protein, human; D000099317:Hippo Kinases
keywords: CD4+ lymphocytopenia; MALT lymphoma; haematopoietic stem cell transplantation; rituximab