T cell lymphoproliferative disorder following bone marrow transplantation for severe aplastic anemia
Journal
Bone Marrow Transplantation
Journal Volume
26
Journal Issue
8
Pages
893-897
Date Issued
2000
Author(s)
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~-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~-2, and ALG 15 mg/kg once daily i.v. on days -4~-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 γ-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.
SDGs
Other Subjects
azathioprine; cyclophosphamide; cyclosporin A; immunosuppressive agent; methotrexate; nonsteroid antiinflammatory agent; prednisolone; T lymphocyte receptor; thymocyte antibody; adolescent; aplastic anemia; article; bone marrow transplantation; case report; cheek mucosa; chromosome analysis; clinical feature; disease severity; drug withdrawal; Epstein Barr virus; face edema; Fanconi anemia; fever; fluorescence in situ hybridization; follow up; gene rearrangement; graft versus host reaction; histopathology; human; immunosuppressive treatment; lymph node biopsy; lymphadenopathy; lymphoproliferative disease; male; mouth ulcer; outpatient department; peripheral blood stem cell; plasmacytosis; priority journal; risk factor; skin exfoliation; skin ulcer; stem cell transplantation; T cell lymphoma; T lymphocyte; treatment failure
Publisher
Nature Publishing Group
Type
journal article