中国人成人T细胞白血病/淋巴瘤12例临床分析

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Ｓｏｕｔｈｅｒｎ Ｂｌｏｔ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

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

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 immunofluorescence method. HTLV-1 antibody was detected by indirect immunofluorescence 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 interleukin 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.