Canioni D, Jabado N, MacIntyre E, Patey N, Emile J F, Brousse N
Service d'Anatomie-Pathologique, Hôpital Necker-Enfants Malades, Paris, France.
Histopathology. 2001 Feb;38(2):146-59. doi: 10.1046/j.1365-2559.2001.01039.x.
Lymphoproliferative disorders (LPDs) are a severe complication in primary immunodeficiency and post-transplant patients. In primary immunodeficiency patients, LPDs are not well-known and, thus, we tried to evaluate their distinctive features and to determine prognostic factors predictive of clinical outcome by comparison with LPDs in post-transplant children.
Clinical records and histopathology of 18 LPDs occurring in primary immunodeficieny children were compared with those of 10 LPDs in post-transplant children, together with results of in-situ hybridization for the detection of Epstein-Barr virus (EBV)-RNA and molecular biological techniques. LPDs were frequently extranodal, EBV-associated, and were more commonly pleomorphic in primary immunodeficiency than in post-transplant patients. A low T-cell count and abnormal T-cell function indicated bad prognosis in both groups. Polymorphic LPDs (PLPDs) were most frequent (n = 19), whereas lymphomas were rare (n = 7), and pseudo-tumoral lymphoid hyperplasias (n = 2) were observed only in primary immunodeficiency. Comparative p53/bcl-2 staining revealed a p53 overexpression in lymphomas compared with PLPDs; CD20/CD79a showed a similar staining in lymphomas, whereas PLPD expressed mainly CD20. TCR and IgH rearrangements did not help in distinguishing PLPDs from lymphomas, but detection of IgH clonality by Southern blot indicated poor prognosis, whereas oligoclonality by Southern blot regardless of PCR clonality and especially a polyclonal profile by Southern blot and PCR indicated a relatively good prognosis.
This study documents the pleomorphism of LPDs in primary immunodeficiency compared to post-transplant children, even if some LPDs are similar in both groups (PLPDs). No criteria are useful enough to ascertain the diagnosis of malignancy in this series. Some molecular biological criteria help to predict the clinical outcome which, nevertheless, seems to depend more on the degree of immunosuppression and on T-lymphocyte presence and function.
淋巴增殖性疾病（LPDs）是原发性免疫缺陷患者和移植后患者的严重并发症。在原发性免疫缺陷患者中，LPDs并不为人熟知，因此，我们试图通过与移植后儿童的LPDs进行比较，评估其独特特征并确定预测临床结局的预后因素。
将18例原发性免疫缺陷儿童发生的LPDs的临床记录和组织病理学与10例移植后儿童的LPDs进行比较，并结合原位杂交检测爱泼斯坦-巴尔病毒（EBV）-RNA的结果以及分子生物学技术。LPDs在原发性免疫缺陷中常为结外、与EBV相关，且比移植后患者更常见多形性。低T细胞计数和异常T细胞功能表明两组预后均不佳。多形性LPDs（PLPDs）最为常见（n = 19），而淋巴瘤罕见（n = 7），仅在原发性免疫缺陷中观察到假性肿瘤性淋巴组织增生（n = 2）。p53/bcl-2染色比较显示，与PLPDs相比，淋巴瘤中p53过表达；CD20/CD79a在淋巴瘤中染色相似，而PLPD主要表达CD20。TCR和IgH重排无助于区分PLPDs与淋巴瘤，但Southern印迹法检测IgH克隆性提示预后不良，而无论PCR克隆性如何，Southern印迹法检测到寡克隆性，尤其是Southern印迹法和PCR检测到多克隆性则提示预后相对较好。
本研究记录了与移植后儿童相比，原发性免疫缺陷中LPDs的多形性，即使两组中某些LPDs相似（PLPDs）。在本系列中，尚无足够有用的标准来确诊恶性肿瘤。一些分子生物学标准有助于预测临床结局，然而，临床结局似乎更多地取决于免疫抑制程度以及T淋巴细胞的存在和功能。
