Nasal-type T/NK lymphomas: A clinicopathologic study of 13 cases
Nasal-type T/NK lymphomas: A clinicopathologic study of 13 cases
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DOI：
10.3109/10428190009053547
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发表时间：
2000-09-01
影响因子：
2.6
通讯作者：
Cabanillas, F
中科院分区：
文献类型：
--
作者：
Rodriguez, J;Romaguera, JE;Cabanillas, F
自然杀伤（NK）细胞淋巴瘤，包括鼻和“鼻型”品种，在修订的欧洲美国淋巴瘤（R. E. A.L.）分类.这组疾病在美国和欧洲很少见，但在亚洲和中美洲更常见。它与EB病毒（EBV）有关，其对治疗的反应和预后通常很差。本研究的目的是描述我们在德克萨斯大学M。D.安德森癌症中心（UTMDACC）在过去的14年。1987年至1999年，13例鼻NK细胞淋巴瘤患者在UTMDACC接受治疗。11例患者最初接受了基于阿霉素的化疗，伴或不伴放疗。1例患者接受干扰素（IFN）-α和维生素A，另1例接受甲氨蝶呤、长春新碱、L-天冬酰胺酶和放疗。中位年龄为44岁（范围15-76岁）;有4名女性和9名男性。所有患者均表现为累及鼻窦区域的局部疾病。所有患者均存在表达CD 2+、CD 3-和CD 56+表面标志物的典型免疫表型以及T受体未重排。8例患者（62%）对治疗有反应; 6例（46%）完全缓解（CR），2例（16%）部分缓解（PR）。5例患者（38%）存活，4例在治疗后1年、2年、3年和9年时无疾病证据（NED），1例患者在发表时存在疾病（AWD）。1例患者在CR时死于同种异体骨髓移植并发症。6例患者的疾病进展至结节部位，包括：睾丸（2例）、中枢神经系统（2例）、肺（1例）、骨髓（2例）、肝脏（2例）、外周血（2例）和皮肤（2例）。总之，对含阿霉素方案的反应不如其他非霍奇金淋巴瘤和类似预后因素的患者。由于90% - 100%的病例与EBV病毒相关，并且预后不良，因此应尝试创新疗法，包括靶向肿瘤表达EBV的免疫疗法，伴或不伴清髓性程序。
Natural Killer (NK) cell lymphomas, which include the nasal and the "nasal type" varieties, are defined as angiocentric lymphomas in the revised European American Lymphoma (R.E.A.L.) classification. This group of diseases is rare in the United States and Europe but is more common in Asia and Central America. It is associated with the Epstein-Barr virus (EBV) and its response to treatment and prognosis are usually very poor. The aim of this study was to describe our experience with 13 patients with angiocentric lymphomas seen at The University of Texas M. D. Anderson Cancer Center (UTMDACC) over the last 14 years. Thirteen patients with a diagnosis of nasal NK cell lymphoma were treated at UTMDACC from 1987 to 1999. Eleven patients were treated initially with doxorubicin based chemotherapy with or without radiotherapy. One patient received interferon (IFN)-alpha and vitamin A and another methotrexate, vincristine, L-Asparaginase, and radiotherapy. The median age was 44 years (range 15-76); there were four women and nine men. All patients presented with local disease involving the sinonasal region. Typical immunophenotypes expressing CD2+, CD3- and CD56+ surface markers as well as non rearrangement of T-receptors were present in all patients. Eight patients (62%) responded to therapy; six (46%) with complete response (CR) and two (16%) with partial response (PR). Five patients (38%) were alive, four with no evidence of disease (NED) at 1, 2, 3, and 9 years after treatment, and one patient was alive with disease (AWD) at the time of publication. One patient died while in CR from complications from allogeneic bone marrow transplant. Six patients had disease progression to extranodal sites including: testis (2), central nervous system (2), lung (1), bone marrow (2), liver (2), peripheral blood (2), and skin (2). In conclusion, the response to doxorubicin-containing regimens is inferior to that of patients with other non-Hodgkin's lymphomas and similar prognostic factors. Because the disease is associated with EBV virus in 90% - 100% of the cases and the prognosis is poor, innovative therapies should be tried including immunotherapy that targets the expression of EBV by the tumor with or without myeloablative procedures.