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[伴有巨核母细胞受累的急性阳性末端脱氧核苷酸转移酶白血病。关于一名婴儿的病例观察]

[Acute positive Tdt leukemia with megakaryoblastic involvement. Apropos of an observation of an infant].

作者信息

Guérin V, Micheau M, Lauroua C, Charrin J L, Dufillot D, Guillard J M, Boisseau M R

出版信息

Nouv Rev Fr Hematol (1978). 1984;26(5):329-33.

PMID:6390337
Abstract

After observation of a 10 month-old infant hospitalised for acute thrombopenia associated with signs of marked radiological hyperostosis, the initial medullograms showed a rich bone marrow free of blast cells. Subsequent complications of the thrombopenia were anemia, neutropenia and circulating blastosis. Marrow aspirations at various sites were unsuccessful. X-rays showed a worsening of the conditions and the child suffers attacks of intense pain. Ultrastructural cytochemistry and Tdt disclosed by immunofluorescence showed that the majority of blasts possessed Tdt and 2-3% were PPO positive. The presence of these 2 markers may correspond either to the presence of a promegakaryoblastic leukemia with aberrant Tdt, or to the coexistence of 2 types of blasts cells.

摘要

在观察一名因急性血小板减少症住院且伴有明显放射学骨质增生迹象的10个月大婴儿后,最初的骨髓造影显示骨髓丰富,无原始细胞。血小板减少症随后出现的并发症是贫血、中性粒细胞减少和循环原始细胞血症。在不同部位进行骨髓穿刺均未成功。X线显示病情恶化,患儿遭受剧烈疼痛发作。超微结构细胞化学和免疫荧光显示的末端脱氧核苷酸转移酶(Tdt)表明,大多数原始细胞具有Tdt,2%-3%为过氧化物酶(PPO)阳性。这两种标志物的存在可能要么对应于伴有异常Tdt的原巨核细胞白血病的存在,要么对应于两种类型原始细胞的共存。

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