Costes-Martineau V, Delfour C, Obled S, Lamant L, Pageaux G-P, Baldet P, Blanc P, Delsol G
Department of Pathology, Hôpital Gui de Chauliac, 34295 Montpellier, France.
J Clin Pathol. 2002 Nov;55(11):868-71. doi: 10.1136/jcp.55.11.868.
Most post transplantation lymphoproliferative disorders (PTLDs) are Epstein-Barr virus (EBV) associated B cell proliferations. We report a case of aggressive anaplastic large cell lymphoma expressing the anaplastic lymphoma kinase (ALK) protein in a 58 year old man who had previously undergone liver transplantation. A definite diagnosis was not possible on histopathological examination. Immunostaining clearly showed a predominant population of small irregular lymphocytes, admixed with large cells strongly positive for CD30, epithelial membrane antigen, and the ALK protein. Neoplastic cells were of the T/cytotoxic phenotype. In situ hybridisation with EBV encoded early RNA probes showed only a few scattered positive non-neoplastic small lymphocytes. Polymerase chain reaction analysis of immunoglobulin and T cell receptor rearrangements was negative. The NPM-ALK fusion transcript associated with the t(2;5) translocation was detected by reverse transcription polymerase chain reaction. A review of the literature revealed 76 cases of T cell PTLD, showing a broad spectrum of morphological features and clinical behaviour. Most of these cases were EBV negative (61 of 76) and occurred after renal transplantation (48 of 76). To our knowledge, this is the first case of ALK positive lymphoma occurring in the setting of organ transplantation. This observation stresses the need for accurate immunostaining for diagnosing this rare, apparently aggressive, lymphoma in immunosuppressed patients.
大多数移植后淋巴细胞增生性疾病（PTLD）是与爱泼斯坦-巴尔病毒（EBV）相关的B细胞增殖性疾病。我们报告一例58岁男性患者，其患有侵袭性间变性大细胞淋巴瘤，表达间变性淋巴瘤激酶（ALK）蛋白，该患者曾接受肝移植。组织病理学检查无法明确诊断。免疫染色清楚地显示主要为小的不规则淋巴细胞群体，混有对CD30、上皮膜抗原和ALK蛋白呈强阳性的大细胞。肿瘤细胞呈T/细胞毒性表型。用EBV编码的早期RNA探针进行原位杂交仅显示少数散在的阳性非肿瘤性小淋巴细胞。免疫球蛋白和T细胞受体重排的聚合酶链反应分析为阴性。通过逆转录聚合酶链反应检测到与t(2;5)易位相关的NPM-ALK融合转录本。文献回顾显示有76例T细胞PTLD病例，呈现出广泛的形态学特征和临床行为。这些病例大多数为EBV阴性（76例中的61例），且发生在肾移植后（76例中的48例）。据我们所知，这是首例在器官移植背景下发生的ALK阳性淋巴瘤病例。这一观察结果强调了在免疫抑制患者中对这种罕见的、明显具有侵袭性的淋巴瘤进行准确免疫染色诊断的必要性。
