5例不典型嗜人T细胞病毒Ⅰ型相关性成人T细胞白血病/淋巴瘤的发现

Discovery of 5 cases of atypical human T-cell lymphotropic virus type-Ⅰ associated adult T-cell leukemia/lymphoma

成人 T 细胞白血病/淋巴瘤(ATLL)至今国内仅发现10余例病人。最近连续发现了5例不典型嗜人 T 细胞病毒Ⅰ型(HTLV-Ⅰ)相关性 ATLL,其中男2例,女3例,平均年龄49.6岁;汉族3例,满族2例(为同一家族成员)。除1例出生在沈阳外,其余出生在北京。均未发现高钙血症。常规血涂片不易发现花细胞,用 CytospinⅡ做外周血单个核细胞浓缩离心甩片易见花细胞和大量异常淋巴细胞。间接免疫荧光法检测 HTLV-Ⅰ抗体4例中2例阳性,聚合酶链反应技术证实在所有5例病人肿瘤细胞中均存在HTLV-Ⅰ前病毒 DNA 的整合。急性型4例,淋巴瘤型1例。4例病人已死亡,生存期10天至不足6个月;1例急性型已存活8个月以上。从以上结果提出如下假设:在中国特别是某些城市 ATLL 并非罕见,提高对不典型临床表现的认识,改进检测技术和常规检测 T 细胞淋巴增殖性疾病病人 HTLV-Ⅰ抗体和前病毒 DNA,将会发现更多的病人。

Only more than 10 cases of adult T-cell leukemia/lymphoma(ATLL)have been reported in this coun- try up to now.Recently,5 cases of atypical HTLV-Ⅰ associated ATLL were consecutively found.There were 2 male and 3 female patients whose age were 49.6 years old in average,while 3 patients were of Han and 2 were of Man nation- alities,the latter 2 patients being the members of the same family.All patients were born in Beijing except one in Shenyang.No hypercalcemia was found in all these cases.Flower cells and abnormal lymphoeytes were not easily found in routine blood smears,but it was easily found these cells in the smears of centrifugated and concentrated peripheral blood mononuclear cells using Cytospin Ⅱ.HTLV-Ⅰ antibody was positive in 2 of 4 cases by using indirect immuno- fuorescence,and the presence of HTLV-Ⅰ proviral DNA integration in tumor cells was demonstrated in all 5 cases by using polymerase chain reaction technique.Four cases died with survival times from 10 days to less than 6 months,and only one has been alive for more than 8 months.A hypothesis has been proposed from this result that in China especially in some cities ATLL is not very rare,and more patients could be found if the knowlegde about its atypical clinical mani- festation is increased,the detecting techniques are improved and the detection of HTLV-Ⅰ antibody and proviral DNA in T-cell lymphoproliferative disorders is routinely performed.