Morrison V A, Dunn D L, Manivel J C, Gajl-Peczalska K J, Peterson B A
Department of Medicine, University of Minnesota Health Sciences Center, Minneapolis.
Am J Med. 1994 Jul;97(1):14-24. doi: 10.1016/0002-9343(94)90043-4.
To study the histopathologic findings, clinical course, and therapeutic outcome of patients who developed a lymphoproliferative disorder after undergoing solid organ transplantation.
A series of 26 patients who developed a lymphoproliferative disorder after solid organ transplant during a 27-year period were studied.
The 26 patients ranged in age from 6 to 68 years (median 42 years). The lymphoproliferative disorder was diagnosed from 1 to 211 months (median 80 months) after transplantation. The type of transplant was kidney (n = 21), heart or heart-lung (n = 4), or liver (n = 1). Most patients received azathioprine and prednisone, in addition to antilymphocyte globulin or cyclosporine, for post-transplant immunosuppression. Eight patients had lymphoma that could be classified according to the International Working Formulation (IWF-F, IWF-G, IWF-H). Sixteen patients had polymorphic lymphoma, and 2 patients were classified as having polymorphic lymphoid hyperplasia. Patients were staged by the Ann Arbor staging system. Nine patients had stage I disease, 4 stage II, 6 stage III, and 7 stage IV. Central nervous system, lung, or marrow involvement was present in 27%, 23%, and 14% of patients, respectively. In the 17 patients studied, immunophenotype was monoclonal B-cell (n = 12), malignant T-cell (n = 2), or polyclonal B-cell (n = 3). The initial therapeutic approach was generally a reduction in immunosuppression, but, thereafter, the approach to therapy varied. In patients with localized disease, surgical excision and/or involved field radiotherapy were utilized as applicable. For patients with more extensive disease, other approaches such as high-dose acyclovir, combination chemotherapy, or alpha interferon were utilized. Overall, 15 of 26 patients (58%) responded to systemic therapy or were rendered disease-free either by surgery or radiation, including 8 (31%) with a complete remission (CR). Only 3 of 9 patients responded to chemotherapy, whereas 4 of 13 patients responded to acyclovir (including 3 patients who experienced CR). Remission duration ranged from 8 to 122 months (median 32+ months). Twenty-one of 26 patients (81%) have died. Survival ranged from less than 1 to 122 months (median 14 months).
The outcome of patients with post-solid organ transplant lymphoproliferative disorders is poor, and the optimal approach to therapy is not clear. Newer therapeutic approaches are thus needed to improve the outcome of these patients.
研究实体器官移植后发生淋巴增殖性疾病患者的组织病理学表现、临床病程及治疗结果。
对27年间一系列26例实体器官移植后发生淋巴增殖性疾病的患者进行研究。
26例患者年龄在6岁至68岁之间（中位年龄42岁）。淋巴增殖性疾病在移植后1个月至211个月（中位时间80个月）被诊断出来。移植类型为肾脏（n = 21）、心脏或心肺联合移植（n = 4）或肝脏移植（n = 1）。大多数患者在移植后免疫抑制治疗中除使用抗淋巴细胞球蛋白或环孢素外，还接受了硫唑嘌呤和泼尼松治疗。8例患者的淋巴瘤可根据国际工作分类法（IWF - F、IWF - G、IWF - H）进行分类。16例患者有多形性淋巴瘤，2例患者被分类为多形性淋巴样增生。患者采用Ann Arbor分期系统进行分期。9例患者为I期疾病，4例为II期，6例为III期，7例为IV期。分别有27%、23%和14%的患者出现中枢神经系统、肺部或骨髓受累。在研究的17例患者中，免疫表型为单克隆B细胞（n = 12）、恶性T细胞（n = 2）或多克隆B细胞（n = 3）。初始治疗方法通常是减少免疫抑制，但此后治疗方法各不相同。对于局限性疾病患者，视情况采用手术切除和/或受累野放疗。对于疾病范围更广的患者，则采用其他方法，如大剂量阿昔洛韦、联合化疗或α干扰素。总体而言，26例患者中有15例（58%）对全身治疗有反应，或通过手术或放疗达到无病状态，其中8例（31%）完全缓解（CR）。9例接受化疗的患者中只有3例有反应，而13例接受阿昔洛韦治疗的患者中有4例有反应（包括3例完全缓解的患者）。缓解持续时间为8个月至122个月（中位时间32 + 个月）。26例患者中有21例（81%）死亡。生存期从不到1个月至122个月不等（中位时间14个月）。
实体器官移植后淋巴增殖性疾病患者的预后较差，最佳治疗方法尚不清楚。因此需要更新的治疗方法来改善这些患者的预后。
