DIAGNOSTIC-CRITERIA AND CLASSIFICATION OF CLINICAL SUBTYPES OF ADULT T-CELL LEUKEMIA-LYMPHOMA - A REPORT FROM THE LYMPHOMA-STUDY-GROUP (1984-87)
DIAGNOSTIC-CRITERIA AND CLASSIFICATION OF CLINICAL SUBTYPES OF ADULT T-CELL LEUKEMIA-LYMPHOMA - A REPORT FROM THE LYMPHOMA-STUDY-GROUP (1984-87)
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DOI：
10.1111/j.1365-2141.1991.tb08051.x
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发表时间：
1991-11-01
影响因子：
6.5
通讯作者：
SHIMOYAMA, M
中科院分区：
文献类型：
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作者：
SHIMOYAMA, M
提出以下诊断标准，将 HTLV-1 相关成人 T 细胞白血病淋巴瘤 (ATL) 的四种临床亚型分类： (1) 冒烟型、PB 中 5% 或以上 T 细胞性质异常淋巴细胞、正常淋巴细胞水平 (< 4 x 10(9)/l)、无高钙血症 (校正钙水平 < 2.74) mmol/l），乳酸脱氢酶（LDH）值达正常上限的1.5倍，无淋巴结肿大，无肝、脾、中枢神经系统（CNS）、骨和胃肠道受累，无腹水和胸腔积液。可能存在皮肤和肺部病变。如果 PB 中异常 T 淋巴细胞少于 5%，则应至少存在一种经组织学证实的皮肤和肺部病变。 (2)慢性型，绝对淋巴细胞增多(4×10(9)/l以上)，T淋巴细胞增多超过3.5×10(9)/l，LDH值达正常上限2倍，无高钙血症，无中枢神经系统、骨和胃肠道受累，无腹水、胸腔积液。可能存在淋巴结肿大以及肝、脾、皮肤和肺受累，大多数情况下PB中可见5%或更多的异常T淋巴细胞。 (3)淋巴瘤型，无淋巴细胞增多，异常T淋巴细胞在1%或以下，组织学证实的淋巴结肿大，伴或不伴结外病变。 (4)急性型，剩余的ATL患者通常具有白血病表现和肿瘤病变，但不属于其他三种类型中的任何一种。按此标准对1983年至1987年新诊断的818例ATL患者进行分析，平均年龄57岁。其中有 448 名男性和 370 名女性，其中 253 例仍存活，诊断后中位随访时间为 13.3 个月，565 例死亡，中位生存时间 (MST) 为 5.4 个月。 MST为急性型6.2个月，淋巴瘤型10.2个月，慢性型24.3个月，阴燃型尚未达到。预计急性型2年和4年生存率为16.7%和5.0%，淋巴瘤型为21.3%和5.7%，慢性型为52.4%和26.9%，阴燃型为77.7%和62.8%。描述了每种临床亚型的独特临床特征和实验室检查结果。
The following diagnostic criteria are proposed to classify four clinical subtypes of HTLV-1 associated adult T-cell leukaemia-lymphoma (ATL): (1) Smouldering type, 5% or more abnormal lymphocytes of T-cell nature in PB, normal lymphocyte level (< 4 x 10(9)/l), no hypercalcaemia (corrected calcium level < 2.74 mmol/l), lactate dehydrogenase (LDH) value of up to 1.5 x the normal upper limit, no lymphadenopathy, no involvement of liver, spleen, central nervous system (CNS), bone and gastrointestinal tract, and neither ascites nor pleural effusion. Skin and pulmonary lesion(s) may be present. In case of less than 5% abnormal T-lymphocytes in PB, at least one of histologically-proven skin and pulmonary lesions should be present. (2) Chronic type, absolute lymphocytosis (4 x 10(9)/l or more) with T-lymphocytosis more than 3.5 x 10(9)/l, LDH value up to twice the normal upper limit, no hypercalcaemia, no involvement of CNS, bone and gastrointestinal tract, and neither ascites nor pleural effusion. Lymphadenopathy and involvement of liver, spleen, skin, and lung may be present, and 5% or more abnormal T-lymphocytes are seen in PB in most cases. (3) Lymphoma type, no lymphocytosis, 1% or less abnormal T-lymphocytes, and histologically-proven lymphadenopathy with or without extranodal lesions. (4) Acute type, remaining ATL patients who have usually leukaemic manifestation and tumour lesions, but are not classified as any of the three other types. A total of 818 ATL patients with a mean age of 57 years, newly diagnosed from 1983 to 1987, were analysed by this criteria. There were 448 males and 370 females, and 253 were still alive with a median follow-up time of 13.3 months from diagnosis, while 565 were dead with a median survival time (MST) of 5.4 months. MST was 6.2 months-for acute type, 10.2 months for lymphoma type, 24.3 months for chronic type, and not yet reached for smouldering type. Projected 2- and 4-year survival rates were 16.7% and 5.0% for acute type, 21.3% and 5.7% for lymphoma type, 52.4% and 26.9% for chronic type, 77.7% and 62.8% for smouldering type, respectively. Distinct clinical features and laboratory findings of each clinical subtype are described.