title: T cell lymphoproliferative disorder following bone marrow transplantation for severe aplastic anemia.
abstract: Post-transplant lymphoproliferative disorder (PTLD) is uncommonly of T cell origin, especially following BMT. We describe a 13-year-old boy with severe aplastic anemia (SAA) and no evidence of Fanconi's anemia who underwent BMT at 11 years of age using CY 10 mg/kg once daily i.v. on days -5, -4, antilymphocyte globulin (ALG) 30 mg/kg once daily i.v. on days -5 approximately -3 and CsA from day -1 as conditioning. The BMT failed and he received a further peripheral blood stem cell transplant (PBSCT) 240 days after BMT. Conditioning was with CY 50 mg/kg once daily i.v. on days -5 approximately -2, and ALG 15 mg/kg once daily i.v. on days -4 approximately -2. GVHD prophylaxis included CsA and MTX. Engraftment was later confirmed by cytogenetic studies. Desquamation and ulcers of the oral mucosa and mouth angle developed in the 13th month post PBSCT. A buccal mucosa biopsy on day +524 revealed only plasmacytosis. Immunosuppressants were discontinued at that point. Generalized lymphadenopathy, prolonged fever (waxing and waning) and facial swelling developed in the 18th month post PBSCT. A neck lymph node biopsy on day +601 showed T cell lymphoma of diffuse large cell type with monoclonal TCR gamma-chain gene rearrangement. A FISH study showed that the malignant T cells were of recipient origin. EBV in situ hybridization was negative. He did not receive further treatment apart from discontinuation of immunosuppressants. He was followed up in our out-patient clinic and showed good performance 1170 days post PBSCT. We speculate that a different mechanism was operating in the pathogenesis of T cell lymphoma in this case. Risk factors include SAA and two transplants, conditioned with CY and ALG, long term use of CsA and treatment with azathioprine.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital, Taipei.
authors.forename: L C
authors.initials: LC
authors.lastname: Wang
authors.forename: M Y
authors.initials: MY
authors.lastname: Lu
authors.forename: J
authors.initials: J
authors.lastname: Yu
authors.forename: S T
authors.initials: ST
authors.lastname: Jou
authors.forename: I P
authors.initials: IP
authors.lastname: Chiang
authors.forename: K H
authors.initials: KH
authors.lastname: Lin
authors.forename: D T
authors.initials: DT
authors.lastname: Lin
mesh_terms: D000293:Adolescent; D000741:Anemia, Aplastic / Q000628:therapy*; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D020031:Epstein-Barr Virus Infections / Q000150:complications; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000628:therapy; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology*