CD56(+) NK lymphomas: Clinicopathological features and prognosis
CD56(+) NK lymphomas: Clinicopathological features and prognosis
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DOI：
10.1046/j.1365-2141.1997.1462962.x
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发表时间：
1997-06-01
影响因子：
6.5
通讯作者：
Ho, FCS
中科院分区：
文献类型：
--
作者：
Kwong, YL;Chan, ACL;Ho, FCS
表面分子CD56标志着一种可能源于自然杀伤(NK)细胞的恶性淋巴瘤。我们对24例CD56(+)NK淋巴瘤/白血病进行了回顾性分析，以确定其临床、病理和预后特征。本文对56例鼻淋巴瘤和204例初诊外周T细胞淋巴瘤进行了回顾性分析。为了专门检查可能起源于NK的淋巴瘤，只分析了CD3表面表达阴性但CD56阳性的淋巴瘤。确诊24例。受累部位依次为鼻部(n=18)、腭部(n=1)、结部(n=1)和多器官(n=4)。在临床上，局限于一个解剖部位的患者(n=20)，大多数症状局限于鼻子，早期比例较高(I：91%；IV：9%)。这些病例中的任何一例都没有涉及骨髓。然而，表现为多器官受累的患者(n=4)表现不同。均表现为全血细胞减少、肝脾肿大、骨髓浸润并吞噬血细胞。1例出现白血期。联合化疗可使75%的局限性疾病患者完全缓解，但仅有25%的患者多器官受累。局限性病变的中位生存期为12个月，多脏器移植组为2个月(P=0.06)，无瘤生存期明显优于多脏器移植组(P<；0.01)。我们得出结论，CD56(+)NK淋巴瘤可以分为两种主要的疾病表现形式：局限性(主要是鼻腔)和多器官受累。每种肿瘤都有不同的临床病理和预后特征。常规化疗似乎对大多数患者无效，需要创新的治疗方式来改善结果。
The surface molecule CD56 marks a category of malignant lymphoma of putative natural killer (NK) cell origin. We conducted a retrospective analysis of 24 cases of CD56(+) NK lymphoma/leukaemia to define the clinico-pathologic and prognostic features of this specific group of lymphomas. 56 cases of nasal lymphomas and 204 cases with an initial diagnosis of peripheral T-cell lymphoma were retrospectively analysed. To specifically examine lymphomas of putative NK origin, only those that were negative for surface expression of CD3 but positive for CD56 were analysed. 24 cases were identified. The initial predominant sites of involvement were nasal (n = 18), palate (n = 1), nodal (n = 1) and multi-organ (n = 4). Clinically, in patients with disease localized to one anatomical site (n = 20), most had symptoms confined to the nose, with a high percentage in early stage (I: 91%; IV: 9%). The marrow was not involved in any of these cases. However, patients with multi-organ involvement at presentation (n = 4) behaved differently. All presented acutely with pancytopenia, hepatosplenomegaly, and marrow infiltration with haemophagocytosis. A leukaemic phase was observed in one case. Anthracycline containing combination chemotherapy resulted in complete remission in 75% of patients with localized disease, but only in 25% with multi-organ involvement. The median survival of patients with localized disease was 12 months, compared with 2 months in the multi-organ group (P = 0.06); the disease-free survival was significantly better in the former (P < 0.01). The overall median survival of all patients was still poor at 11 months.We conclude that CD56(+) NK lymphomas could be divided into two main patterns of disease presentations: localized (predominantly nasal), and multi-organ involvement. Each has different clinicopathologic and prognostic features. Conventional chemotherapy appeared ineffective for the majority of patients, and innovative treatment modalities are needed to improve outcome.