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骨髓纤维化和髓样化生中的淋巴细胞白血病转化(淋巴细胞危象)

Lymphoblastic leukemic transformation (lymphoblastic crisis) in myelofibrosis and myeloid metaplasia.

作者信息

Polliack A, Prokocimer M, Matzner Y

出版信息

Am J Hematol. 1980;9(2):211-20. doi: 10.1002/ajh.2830090209.

Abstract

Acute lymphoblastic leukemia (ALL) developing in myelofibrosis (MF) and myeloid metaplasia (MM) is reported in two patients. In both cases, the clinical course of the "blastic crisis" was rapidly progressive with little response to chemotherapy. The circulating cells were readily identified as lymphoblasts on the basis of cytology, cytochemistry, immunologic studies, and ultrastructure. In one of the cases, 40% of cells had T-cell markers and all cells contained paranuclear acid phosphatase. In the second case, cells had a "Burkitt-like appearance, contained multiple cytoplasmic vacuoles positive for oil red O, and one-third of them had B-cell markers. The development of lymphoblastic crisis in MF and MM occurs rarely, is analogous to blastic transformation in chronic granulocytic leukemia, and supports the hypothesis that myeloproliferative disorders originate from pluripotent hematopoietic stem cells.

摘要

两名患者报告了在骨髓纤维化(MF)和髓样化生(MM)中发生的急性淋巴细胞白血病(ALL)。在这两个病例中,“原始细胞危象”的临床病程进展迅速,对化疗反应甚微。根据细胞学、细胞化学、免疫学研究和超微结构,循环细胞很容易被鉴定为原始淋巴细胞。在其中一个病例中,40%的细胞具有T细胞标志物,所有细胞均含有核旁酸性磷酸酶。在第二个病例中,细胞具有“伯基特样外观”,含有多个油红O阳性的细胞质空泡,其中三分之一具有B细胞标志物。MF和MM中原始淋巴细胞危象的发生很少见,类似于慢性粒细胞白血病中的原始细胞转化,并支持骨髓增殖性疾病起源于多能造血干细胞的假说。

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