Tey Siok-Keen, Marlton Paula V, Hawley Carmel M, Norris Debra, Gill Devinder S
Department of Clinical Hematology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.
Am J Hematol. 2008 Apr;83(4):330-3. doi: 10.1002/ajh.21062.
Hepatosplenic T-cell lymphoma (HSTL) is an aggressive lymphoma. In post-transplant immunosuppressed patients, HSTL is usually rapidly fatal. We report successful treatment of post-transplant HSTL in a 50-year-old renal allograft recipient by reducing immunosuppression and using intensive chemotherapy consisting of alternating cycles of HyperCVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and MTX/HiDAC (methotrexate, Ara-C). Remission is ongoing at 8+ years. Literature review identified another 20 cases of HSTL in solid organ transplant recipients: median survival was 4 months; no other patients survived beyond 12 months. Bone marrow involvement was universal, but changes were often subtle: 6 of 12 cases had nondiagnostic examinations earlier on. High index of suspicion may lead to more timely diagnosis of this uncommon form of post-transplant lymphoproliferative disorder, and treatment with intensive chemotherapy such as HyperCVAD may be curative.
肝脾T细胞淋巴瘤(HSTL)是一种侵袭性淋巴瘤。在移植后免疫抑制的患者中,HSTL通常会迅速致命。我们报告了一名50岁肾移植受者的移植后HSTL通过减少免疫抑制并使用由交替周期的HyperCVAD(环磷酰胺、长春新碱、阿霉素和地塞米松)和MTX/HiDAC(甲氨蝶呤、阿糖胞苷)组成的强化化疗成功治疗的案例。缓解状态持续了8年多。文献回顾发现实体器官移植受者中另有20例HSTL病例:中位生存期为4个月;没有其他患者存活超过12个月。骨髓受累普遍存在,但变化往往很细微:12例中有6例早期检查未确诊。高度怀疑可能会导致更及时地诊断这种罕见的移植后淋巴增殖性疾病形式,并且使用如HyperCVAD这样的强化化疗进行治疗可能会治愈。