中国人成人T细胞白血病/淋巴瘤12例临床分析

Clinical analysis of 12 cases of Chinese patients with adult T cell leukemia/lymphoma

目的探讨中国人成人Ｔ细胞白血病／淋巴瘤（ＡＬＴＴ）临床特征、鉴别诊断和与嗜人Ｔ细胞病毒Ⅰ型（ＨＴＬＶⅠ）感染的关系。方法外周血淋巴细胞形态学检查用离心涂片法，淋巴细胞免疫分型用间接免疫荧光法，ＨＴＬＶⅠ前病毒ＤＮＡ检测用聚合酶链反应（ＰＣＲ）法并经ＳｏｕｔｈｅｒｎＢｌｏｔ证实。结果１２例病人临床表现依次为发热１０例、淋巴结肿大８例、皮肤损害７例、脾、肝大７例、黄疸３例，个别有胸水和肺侵犯。外周血花瓣状淋巴细胞在常规血涂片中不易发现，而离心涂片均可发现。可见贫血、血小板减少、全血细胞减少、骨髓增生异常和嗜酸细胞增多，未见高钙血症。ＨＴＬＶⅠ抗体和前病毒ＤＮＡ阳性者分别为６／１０例和９／１２例。急性型９例，淋巴瘤型３例。所有病人开始均误诊为其他疾病，首发表现分别为皮肤损害、淋巴结肿大、骨髓增生异常、进行性肝肿大和顽固性胸水。合并症有未经病原菌证实的感染、链球菌感染、消化道出血、急性肾功能衰竭、急性呼吸衰竭和脑出血。７例死亡，３例存活，２例失访。结论ＡＴＬＬ在我国可能并非罕见，应提高对中国人ＡＴＬＬ的认识。

Objective To define the clinical characteristics and differential diagnosis in Chinese patients with adult T cell leukemia/lymphoma (ATLL) and its relation to human T lymphotropic virus type 1 (HTLV 1) infection. Methods Lymphocyte morphology in peripheral blood was examined in smears by Cytospin Ⅱ. Lymphocyte immunophenotyping was carried out by indirect immunoflourence method. HTLV 1 antibody was detected by indirect immunoflourence method, proviral DNA detected by PCR method and PCR products demonstrated by Southern Blot analysis. Results The clinical manifestations in these 12 patients were fever (10/12), lymphadenopathy (8/12), skin changes (7/12), spleen and liver enlargement (7/12), jaundice (3/12), pleural effusion (1/12) and lung invasion (1/12). Patul like cells were not easily found in routine blood smears, but easily found with Cytospin Ⅱ (10/10). There was increase in lactic dehydrogenase (8/9) and soluble interlukin 2 receptor (9/9). Anemia (7/12), thrombocytopenia (6/12), pancytopenia (2/12), myelodysplasia (4/12) and eosinophilia (8/12) can be found, but no hypercalcemia. HTLV 1 antibody and proviral DNA were positive in 6/10 cases and 9/12 cases respectively. Nine cases were classified as acute type and three as lymphoma type. All patients were misdiagnosed as other diseases at the beginning. The initial presentations were skin changes (4/12), lymphadenopathy (3/12), myelodysplasia (3/12), progressive liver enlargement (1/12) and refractory pleural effusion (1/12). The complications were infections by undetermined pathogens (6/12), Streptococcal infection (1/12), gastrointestinal bleeding (3/12), acute renal failure (3/12), acute respiratory failure (2/12) and cerebral hemorrhage (1/12). Seven patients died, three still survive, and two are out of contact. Conclusion ATLL may not be very rare in China. It is necessary to deepen the recognition of ATLL in Chinese.