title: A unique description of stage IV extranodal marginal zone lymphoma (EMZL) in an adolescent associated with gamma heavy chain disease.
abstract: Extranodal Marginal zone lymphoma (EMZL) is a rare, usually localized disease in children. Advanced stage EMZL in adults is considered incurable, with prolonged remissions after chemotherapy. Gamma heavy chain disease (γHCD) is a rare disease of adults associated with lympho-proliferative processes with no comparable reports in children. A case of stage-IV EMZL with γHCD in an adolescent is discussed including treatment with Bendamustine plus Rituximab. The patient remains disease free 18 months from diagnosis. This case highlights necessity for careful diagnostic work-up to identify indolent lymphomas in children which may respond to less toxic chemotherapy than used for common pediatric lymphomas.
authors: Department of Pediatrics, Division of Pediatric Hematology Oncology, University of Illinois at Chicago, Chicago, Illinois; Department of Pediatrics, Division of Pediatric Hematology Oncology, Rush University Medical Center, Chicago, Illinois; Department of Pediatrics, Division of Pediatric Hematology Oncology, John. H. Stroger Hospital, Chicago, Illinois.
authors: Nupur
authors: N
authors: Mittal
authors: Bing
authors: B
authors: Zhu
authors: Sujata
authors: S
authors: Gaitonde
authors: Yang
authors: Y
authors: Lu
authors: Mary Lou
authors: ML
authors: Schmidt
mesh_terms: D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D000069461:Bendamustine Hydrochloride; D006362:Heavy Chain Disease / Q000150:complications / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications / Q000188:drug therapy* / Q000473:pathology*; D008297:Male; D009588:Nitrogen Mustard Compounds / Q000008:administration & dosage; D011379:Prognosis; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D009588:Nitrogen Mustard Compounds; D000069283:Rituximab; D000069461:Bendamustine Hydrochloride
keywords: bendamustine-rituximab; extranodal marginal zone lymphoma; gamma heavy chain disease; pediatric NHL