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关于成人T细胞白血病隐匿期的建议——成人T细胞白血病临床症状的多样性——

A proposal for smoldering adult T-cell leukemia--diversity in clinical pictures of adult T-cell leukemia--.

作者信息

Yamaguchi K, Nishimura H, Kawano F, Kohrogi H, Jono M, Miyamoto Y, Takatsuki K

出版信息

Jpn J Clin Oncol. 1983;13 Suppl 2:189-99.

PMID:6603527
Abstract

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly but flared up after several years. Skin lesions appeared in the form of erythema, papules or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was from 0% to 2%. The serum lactic dehydrogenase value was within normal range, and was not associated with hypercalcemia, lymphadenopathy, or hepatosplenomegaly, and bone marrow infiltration was very slight. In most cases, hypergammaglobulinemia was seen, and in one case monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their sera were positive for anti-ATL-associated antigen antibodies. None of them had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness, and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness, and died or cryptococcus meningitis. These cases were clinically and pathologically different from typical ATL cases already reported, and we feel it necessary to make distinctions from the viewpoints of prognosis and treatment. In discussing these cases, we compared smoldering ATL with typical ATL, and deliberated upon the causes of both.

摘要

我们观察了5例成人隐匿型T细胞白血病(ATL)患者,他们以皮肤病变作为先兆症状。病情发展缓慢,但数年后会突然加重。皮肤病变表现为红斑、丘疹或结节。ATL细胞对皮肤的浸润较轻,外周血中ATL细胞的比例为0%至2%。血清乳酸脱氢酶值在正常范围内,与高钙血症、淋巴结病或肝脾肿大无关,骨髓浸润也非常轻微。大多数病例可见高球蛋白血症,1例观察到单克隆高球蛋白血症。所有5例患者都生活在ATL流行地区,他们的血清抗ATL相关抗原抗体呈阳性。他们均未曾接受过输血。1例患者在患病13年多后发展为典型ATL,死于肾功能不全。另1例患者在患病5年后发展为典型ATL,死于隐球菌性脑膜炎。这些病例在临床和病理上与已报道的典型ATL病例不同,我们认为有必要从预后和治疗的角度进行区分。在讨论这些病例时,我们将隐匿型ATL与典型ATL进行了比较,并探讨了两者的病因。

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