ADULT T-CELL LEUKEMIA - CLINICAL AND HEMATOLOGIC FEATURES OF 16 CASES

ADULT T-CELL LEUKEMIA - CLINICAL AND HEMATOLOGIC FEATURES OF 16 CASES
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DOI:
10.1182/blood.v50.3.481.481
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发表时间:
1977-01-01
期刊:
影响因子:
20.3
通讯作者:
UCHINO, H
UCHINO, H
中科院分区:
医学1区
文献类型:
--
作者:
UCHIYAMA, T;YODOI, J;UCHINO, H

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日本报道了16例成人T细胞白血病患者的临床和血液学研究。日本各种淋巴增生性疾病的发病率与西方国家不同。用抗人T(ATS)和B(骨髓源性)细胞(ABS)的抗血清,以及常用的T和B细胞标记物(E[绵羊红细胞]花环、EAC[红细胞、抗体、补体]花环和表面免疫球蛋白)对白血病细胞进行细胞毒试验。临床和血液学表现的特点如下:起病于成人;亚急性或慢性白血病,进展迅速,终末期;白血病细胞被ATS杀死并形成E玫瑰花环;白血病细胞形态不单调,细胞多见,核深凹或分叶;皮肤多发(9例);常见的淋巴结病和肝脾肿大;无纵隔肿块;最显著的发现是患者的出生地聚集,即13例出生在九州的患者。这些病例与其他亚急性或慢性成人T细胞恶性肿瘤的关系,如T细胞起源的慢性淋巴细胞白血病、具有T细胞特性的前淋巴细胞白血病。讨论了Sezary综合征和真菌样肉芽肿。
Clinical and hematologic studies of 16 adult patients whose leukemic cells had T [thymus-derived] cell markers are reported from Japan, where the incidence of various lymphoproliferative diseases differs from that in Western countries. Leukemic cells were studied by cytotoxicity tests with specific antisera against human T (ATS) and B [bone marrow-derived] cells (ABS) in addition to the usual T- and B-cell markers (E [sheep erythrocyte] rosette, EAC [erythrocyte, antibody, complement] rosette and surface immunoglobulins). Characteristics of the clinical and hematologic findings were as follows: onset in adulthood; subacute or chronic leukemia with rapidly progressive terminal course; leukemic cells killed by ATS and forming E rosettes; leukemic cells not morphologically monotonous and frequent cells with deeply indented or lobulated nuclei; frequent skin involvement (9 patients); common lymphadenopathy and hepatosplenomegaly; no mediastinal mass; and, the most striking finding, the clustering of the patients'' birthplaces, namely, 13 patients born in Kyushu. The relationship between these cases and other subacute or chronic adult T cell malignancies such as chronic lymphocytic leukemia of T cell origin, prolymphocytic leukemia with T cell properties. Sezary syndrome and mycosis fungoides is discussed.