Clinical and pathological features of posttransplantation lymphoproliferative disorders presenting with skin involvement in 4 patients
Clinical and pathological features of posttransplantation lymphoproliferative disorders presenting with skin involvement in 4 patients
复制标题
DOI：
10.1001/archderm.140.9.1140
复制
发表时间：
2004-09-01
影响因子：
--
通讯作者：
Kim, YH
中科院分区：
文献类型：
--
作者：
Beynet, DP;Wee, SA;Kim, YH
背景：移植后淋巴增生性疾病(PTLDS)是指实质器官或异基因骨髓移植受者发生的淋巴增殖性疾病。它们在临床和病理上是不同的，范围从多克隆增生性病变到恶性淋巴瘤。虽然结外受累在PTLD中很常见，但皮肤表现很少见，仅有19例报道。观察：我们描述了4例PTLD的皮肤表现。所有患者都有相对较晚的PTLD(移植后1年)，从器官移植到肿瘤诊断的中位数为8年。皮肤病变的范围、数量和解剖位置从局部的斑块到广泛的结节不等。在诊断为PTLD时，所有患者均未出现全身症状。病理表现从浆细胞性增生到单形性PTLD。原位杂交检测3例可评价组织中的EB病毒信使RNA。所有患者都减少了免疫抑制治疗，并接受了其他个体化治疗。中位随访期为2.5年。在最近的随访中，3名患者完全缓解，1名患者残留疾病。结论：在本研究中，皮肤上出现的PTLD皮损对治疗有效。尽管移植后发生的时间相对较晚，但这些病例中的大多数都是EB病毒阳性。与其他皮肤淋巴瘤一样，以皮肤受累为主的PTLDS预后相对较好。
Background: Posttransplantation lymphoproliferative disorders (PTLDs) are lymphoid proliferations that can develop in recipients of solid organ or allogeneic bone marrow transplants. They are clinically and pathologically heterogeneous and range from polyclonal hyperplastic lesions to malignant lymphomas. Although extranodal involvement in PTLD is common, cutaneous presentation is rare, with only 19 cases reported previously.Observations: We describe 4 patients with cutaneous presentations of PTLD. All patients had relatively late-onset PTLD (>1 year after transplantation) with a median of 8 years from organ allograft to tumor diagnosis. The extent, number, and anatomic location of skin lesions varied from a localized patch to widespread nodules. None of the patients exhibited systemic symptoms at the time of PTLD diagnosis. Pathological findings ranged from plasmacytic hyperplasia to monomorphic PTLD. In situ hybridization detected Epstein-Barr virus messenger RNA in all 3 cases with evaluable tissue. All patients underwent reduction in immunosuppressive therapy and received other individualized treatments. Median follow-up was 2.5 years. At the most recent followup, 3 patients were in complete remission and 1 had residual disease.Conclusions: In this study, PTLD lesions presenting in the skin responded to therapy. Despite their relatively late occurrence after transplantation, most of these cases were positive for Epstein-Barr virus. As observed with other cutaneous lymphomas, the PTLDs with predominant skin involvement had a relatively favorable outcome.