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目的回顾性分析造血干细胞移植(HSCT)后淋巴细胞增殖性疾病(PTLD)发生率及临床特点,以提高对本病的鉴别诊断能力。方法选自2002年8月至2006年10月行异基因-HSCT患者585例。供者来源分别为:相合同胞327例,HLA 不合亲源供者208例,非血缘供者44例,脐带血6例。PTLD 确诊依据为病理学检查,包括以器官侵袭和淋巴结结构破坏为特点的各种弥漫性淋巴增殖改变;如果无法获得病理结果,PTLD 的临床诊断依据为:经验性抗生素治疗无效的高热伴淋巴结肿大,伴或不伴 EB 病毒负荷增高,临床能除外其他常见原因的发热。结果 PTLD 发生率在配型相合移植组0.3%(1/327),该例患者为减低预处理剂量的移植,加用了抗胸腺细胞球蛋白(ATG),常规移植的患者无一例发生 PTLD,半相合移植组为3.4%(7/208),非血缘移植组2.3%(1/44);PTLD发生率在应用 ATC 的患者中为3.4%(9/262),高于常规移植不用 ATG 者(0/323,P=0.005);半相合移植和非血缘供者的移植中 PTLD 发生率高于配型相合的常规移植(7/208,1/44,0/323,P=0.036)。诊断 PTLD 9例中4例为病理检查确诊,5例为临床诊断。PTLD 临床表现不一,2例发生在移植半年之后,表现为局部包块或纵隔肿大,病理证实为小淋巴细胞性淋巴瘤;其他7例患者均发生在移植后近期(32～78d),表现为经验性抗生素治疗无效的高热伴颈部淋巴结进行性肿大,4例很快出现呼吸衰竭,1例出现昏迷。9例中1例经减免疫抑制剂和用皮质激素治疗后恢复,1例抗病毒药和供者淋巴细胞输注(DLI)后恢复,其余7例均在短期内疾病进展死亡。结论 HSCT 后 PTLD 是一组进展迅猛、预后极差、临床和病理异质性的疾病,尤其在具备高危因素的患者中更要注意早期识别。
Objective To retrospectively analyze the incidence and clinical features of lymphoproliferative diseases (PTLD) after hematopoietic stem cell transplantation (HSCT) in order to improve the differential diagnosis of this disease. Methods From August 2002 to October 2006 585 cases of allogeneic-HSCT patients. Donor sources were: 327 matched siblings, 208 HLA-matched donors, 44 non-donor and 6 cord blood. PTLD diagnosis is based on pathological examination, including various diffuse lymphatic proliferation changes characterized by organ invasion and structural destruction of lymph nodes; if the pathological findings can not be obtained, the clinical diagnosis of PTLD is based on the following: Experienced antibiotic treatment of hyperthermia with lymph node enlargement Large, with or without EB virus load increased, clinical except for other common causes of fever. Results The incidence of PTLD was 0.3% (1/327) in matched matched transplantation group. This patient was treated with the reduction of pretreatment dose and added anti-thymocyte globulin (ATG). None of the patients with conventional transplantation had PTLD, The incidence of PTLD was 3.4% (7/8) in the semi-coincident transplantation group and 2.3% (1/44) in the non-HLA group, and 3.4% (9/262) in the patients with ATC compared with those without ATG (0/323, P = 0.005). The incidence of PTLD was significantly higher in haploidentical and non-hepatic donors compared with conventional transplantation (7 / 208,1 / 44,0 / 323, P = 0.036). Among the 9 cases diagnosed with PTLD, 4 cases were diagnosed by pathology and 5 cases were diagnosed clinically. The clinical manifestations of PTLD were different. Two cases occurred in six months after transplantation, showing partial mass or mediastinal enlargement and pathological diagnosis of small lymphocytic lymphoma. The other seven cases occurred in the short term after transplantation (32-78 days) The performance of empirical antibiotic treatment of fever with cervical lymph node enlargement, respiratory failure occurred in 4 cases, 1 case of coma. One of nine patients recovered after immunosuppressive therapy and corticosteroid treatment, one patient recovered after antiviral treatment and donor lymphocyte infusion (DLI), and the remaining seven patients all died of disease in a short period of time. Conclusion Post-HSCT PTLD is a group of patients with rapid progression, poor prognosis, clinical and pathological heterogeneity, especially in patients with high risk factors should pay more attention to early recognition.