Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
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Post-transplant lymphoproliferative disorder (PTLD) constitutes a serious complication of allogeneic bone marrow transplantation. We describe a case of PTLD in a twenty-six year-old male treated with bone marrow transplantation for aplastic anemia of unknown cause. The patient received unmanipulated marrow graft from his HLA-matched brother. Fifty-one days post transplant he developed progressive enlargement of cervical lymph nodes, followed by hepatosplenomegaly and generalized lymphadenopathy. Polymorphic PTLD was diagnosed basing on the lymph node histopathology, positive EBV detection, flow cytometry and IgH rearrangement studies proving monoclonality (capillary electrophoresis with ABI PRISM 310 Genetic Analyzer). There was no response to anti-CD20 antibody, cessation of immunosuppression, donor lymphocyte infusion and cytostatic therapy. The patient died on the 65th day of multiple organ failure. We discuss the diagnostics and management of PTLD in the setting of bone marrow transplantation.
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PMID:Fatal post-transplant lymphoproliferative disorder following allogeneic bone marrow transplantation for aplastic anemia. 1201 24