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慢性髓性白血病中的巨核母细胞微巨核细胞危象

Megakaryoblastic micromegakaryocytic crisis in chronic myeloid leukemia.

作者信息

Lingg G, Schmalzl F, Breton-Gorius J, Tabilio A, Schaefer H E, Geissler D, Schweiger M, Kirchmair W

出版信息

Blut. 1985 Oct;51(4):275-85. doi: 10.1007/BF00320522.

DOI:10.1007/BF00320522
PMID:3863678
Abstract

Atypical megakaryoblasts (MKB) or megakaryocytes (MK) are occasionally present in the peripheral blood during the terminal development of chronic myeloid leukemia (CML). We report on a 49-year-old female suffering from Ph1 chromosome-positive CML with typical megakaryoblastic transformation in the peripheral blood and in the bone marrow. The small "blasts" were at the most only slightly larger and were occasionally even smaller than lymphocytes but showed megakaryoblastic or atypical megakaryocytic differentiation. The cytoplasmic cytochemical pattern of the atypical megakaryocytic cells was identical to that of large atypical thrombocytes. Platelet peroxidase was detected upon electron-microscopic (EM) examination. Immunologic characterization disclosed the presence of MK-specific antigens. When cultured in vitro on agar, the blasts transformed spontaneously into large mature MK, exhibiting characteristic cytochemical and immunological patterns. Cytogenetic examination of peripheral blood showed severe abnormalities. The patient did not respond to therapy and died 3 months after manifestation of the blast crisis.

摘要

在慢性髓性白血病(CML)终末期,外周血中偶尔会出现非典型巨核母细胞(MKB)或巨核细胞(MK)。我们报告了一名49岁女性,患有Ph1染色体阳性的CML,外周血和骨髓中出现典型的巨核母细胞转化。这些小“原始细胞”最大仅略大于淋巴细胞,偶尔甚至比淋巴细胞还小,但显示出巨核母细胞或非典型巨核细胞分化。非典型巨核细胞的细胞质细胞化学模式与大型非典型血小板相同。电子显微镜(EM)检查发现了血小板过氧化物酶。免疫特征显示存在MK特异性抗原。当在琼脂上体外培养时,原始细胞自发转化为大型成熟MK,表现出特征性的细胞化学和免疫模式。外周血细胞遗传学检查显示严重异常。患者对治疗无反应,在急变期出现3个月后死亡。

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引用本文的文献

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Acute myelofibrosis in megakaryoblastic leukemia with translocation between chromosomes 8 and 14.
Klin Wochenschr. 1987 Nov 2;65(21):1034-41. doi: 10.1007/BF01726322.
2
Human megakaryocytic progenitor cells.人巨核细胞祖细胞。
Klin Wochenschr. 1987 Apr 1;65(7):297-307. doi: 10.1007/BF01745383.

本文引用的文献

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