Halkos Michael E, Miller Joseph I, Mann Karen P, Miller Daniel L, Gal Anthony A
Cardiothoracic Research Laboratory, Division of Cardiothoracic Surgery, 550 Peachtree Street NE, Atlanta, GA 30308-2225, USA.
Chest. 2004 Dec;126(6):2013-20. doi: 10.1378/chest.126.6.2013.
Posttransplant lymphoproliferative disorders (PTLDs) are rare complications following transplantation. Although organ-specific cases have been reported, primary presentation in the thoracic cavity has not been fully characterized.
Eleven cases of PTLD with a primary thoracic presentation were identified among 3,085 solid-organ transplant patients and 1,662 bone marrow transplant patients from 1990 to 2001.
There were eight men and three women with a mean age of 49 years. Transplanted organs included lungs (three patients), kidneys (three patients), kidney/pancreas (two patients), allogeneic bone marrow (two patients), and heart (one patient). The time to presentation ranged from 1 to 97 months (median time, 8 months). Six patients developed PTLD within 1 year of undergoing transplantation. Pretransplant serology for Epstein-Barr virus (EBV) and cytomegalovirus was negative in 80% and 78% of cases, respectively. Radiographic evaluation revealed mediastinal adenopathy in 45% of patients, and pulmonary parenchymal lesions in 55%. Fifty-five percent of patients also had extrathoracic involvement. Diagnosis was achieved by CT-guided transthoracic needle biopsy in eight patients, and by open biopsy in three patients. Pathologic analysis revealed monomorphic PTLD (ie, diffuse large B-cell lymphoma) in seven patients, polymorphic PTLD in two patients, anaplastic large cell lymphoma in one patient, and Hodgkin lymphoma in one patient. Eighty-four percent of the specimens evaluated for EBV were determined to be positive by in situ hybridization and/or immunohistochemistry. All patients were initially treated with a reduction in immunosuppression therapy, and six patients (55%) received adjuvant chemotherapy. The overall mortality rate was 64%. Four patients died from complications of PTLD (kidney, two patients; heart, one patient; bone marrow, one patient), and three patients (all lung transplant recipients) died from rejection or infectious complications. The median interval from diagnosis to death was 13 months (range, 1 to 42 months).
Thoracic PTLD can occur in any transplant patient and must be regarded as a potentially fatal complication in the immunosuppressed patient. Heart and lung allograft recipients have the worst prognosis because of the mortality that accompanies rejection with subtherapeutic immunosuppression therapy. Earlier diagnosis and improvements in immunosuppression and chemotherapy may improve survival for these inherently high-risk patients.
移植后淋巴组织增生性疾病（PTLDs）是移植后的罕见并发症。尽管已有器官特异性病例的报道，但胸腔原发性表现尚未得到充分描述。
在1990年至2001年的3085例实体器官移植患者和1662例骨髓移植患者中，确定了11例原发性胸腔表现的PTLD病例。
有8名男性和3名女性，平均年龄49岁。移植器官包括肺（3例患者）、肾（3例患者）、肾/胰腺（2例患者）、同种异体骨髓（2例患者）和心脏（1例患者）。出现症状的时间为1至97个月（中位时间为8个月）。6例患者在移植后1年内发生PTLD。移植前针对爱泼斯坦-巴尔病毒（EBV）和巨细胞病毒的血清学检查分别在80%和78%的病例中呈阴性。影像学评估显示45%的患者有纵隔淋巴结肿大，55%的患者有肺实质病变。55%的患者也有胸外受累。8例患者通过CT引导下经胸针吸活检确诊，3例患者通过开放活检确诊。病理分析显示7例患者为单形性PTLD（即弥漫性大B细胞淋巴瘤），2例患者为多形性PTLD，1例患者为间变性大细胞淋巴瘤，1例患者为霍奇金淋巴瘤。通过原位杂交和/或免疫组化评估，84%的EBV标本被确定为阳性。所有患者最初均接受免疫抑制治疗减量，6例患者（55%）接受辅助化疗。总死亡率为64%。4例患者死于PTLD并发症（肾，2例患者；心脏，1例患者；骨髓，1例患者），3例患者（均为肺移植受者）死于排斥反应或感染并发症。从诊断到死亡的中位间隔时间为13个月（范围为1至42个月）。
胸腔PTLD可发生于任何移植患者，必须被视为免疫抑制患者潜在的致命并发症。心脏和肺移植受者预后最差，因为亚治疗性免疫抑制治疗伴随排斥反应会导致死亡。早期诊断以及免疫抑制和化疗的改善可能会提高这些固有高危患者的生存率。
