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向巨核细胞选择性分化的骨髓增殖性疾病。三例尸检病例的临床病理研究。

Myeloproliferative disorders with selective differentiation toward megakaryocytic cells. A clinicopathological study of three autopsy cases.

作者信息

Okayasu I, Kajita A, Takemura T

出版信息

Acta Pathol Jpn. 1975 Mar;25(2):225-39. doi: 10.1111/j.1440-1827.1975.tb00859.x.

DOI:10.1111/j.1440-1827.1975.tb00859.x
PMID:1058628
Abstract

Three autopsy cases of myeloproliferative disorders with selective differentiation toward megakaryocytic cells were reported with a clinicopathological analysis. All of these cases had some common distinctive features; (1) patients, adult of more than 40 years of age, (2) relatively acute fatal course (about 20 to 100 days), (3) continued pancytopenia in the peripheral blood, (4) so-called blast cells, occasionally showing accumulation, according to the length of total duration, in addition to overwhelming predominance of atypical megakaryocytic cells in the bone marrow, and (5) an increased tendency of fibrillosis in the interstitium of the bone marrow corresponding to the more or less prolonged duration of the course. From these facts, they should be compatible with so-called megakaryocytic myelosis. Some relationship between this disorder and myelogenous leukemia, and infiltrative tendency of megakaryocytic cells in the extramedullary organs had also been discussed shortly.

摘要

报告了3例骨髓增殖性疾病尸检病例,这些疾病具有向巨核细胞选择性分化的特点,并进行了临床病理分析。所有这些病例都有一些共同的显著特征:(1)患者为40岁以上成年人;(2)病程相对急性且致命(约20至100天);(3)外周血持续全血细胞减少;(4)所谓的原始细胞,根据总病程长短偶尔会出现聚集,此外骨髓中异常巨核细胞占压倒性优势;(5)随着病程或多或少的延长,骨髓间质纤维化倾向增加。基于这些事实,它们应符合所谓的巨核细胞性骨髓增生症。还简要讨论了这种疾病与骨髓性白血病之间的一些关系,以及巨核细胞在髓外器官的浸润倾向。

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Myeloproliferative disorders with selective differentiation toward megakaryocytic cells. A clinicopathological study of three autopsy cases.向巨核细胞选择性分化的骨髓增殖性疾病。三例尸检病例的临床病理研究。
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