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原发性(特发性)血小板增多症与真性红细胞增多症。骨髓活检切片中骨髓特征的组织形态计量学分析。

Primary (essential) thrombocythemia versus polycythemia vera rubra. A histomorphometric analysis of bone marrow features in trephine biopsies.

作者信息

Thiele J, Zankovich R, Schneider G, Kremer B, Fischer R, Diehl V

机构信息

Institute of Pathology, University of Cologne, Federal Republic of Germany.

出版信息

Anal Quant Cytol Histol. 1988 Oct;10(5):375-82.

PMID:3207462
Abstract

A morphometric analysis of bone marrow biopsies was performed in 25 patients each with clinical diagnoses of primary (essential) thrombocythemia (PTH) and polycythemia vera rubra (P. vera) according to the rigid diagnostic criteria of the Polycythemia Vera Study Group to reveal significant differences in the histomorphologic features between these disorders. In comparison with control specimens of patients without any hematologic disease, megakaryocyte proliferation was most prominent in PTH, even exceeding that of P. vera with concomitant thrombocythemia (11 of 25 cases with a platelet count greater than 600 X 10(9)/L). Moreover, in P. vera there were wide ranges of megakaryocyte sizes, consisting of micro-megakaryocytes as well as giant forms with highly segmented nuclei (four nuclear lobes), which gave the cells a pleomorphic appearance. As compared with the normal bone marrow, the amount of neutrophilic granulopoiesis and erythropoiesis was not significantly increased in PTH, in contrast to P. vera. Similar results were obtainable regarding the density of reticulin (argyrophilic) fibers: a normal content was encountered in the control specimens and PTH, whereas P. vera displayed a minimal-to-slight increase. Finally, the bone marrow of P. vera was totally devoid of stainable iron while hemosiderin deposits were detected in about two-thirds of the patients without hematologic disorders and in PTH. The characteristic differences revealed by this morphometric study may lead to an improvement of the controversial histologic diagnosis in these disorders.

摘要

根据真性红细胞增多症研究组严格的诊断标准,对25例临床诊断为原发性(特发性)血小板增多症(PTH)和真性红细胞增多症(P. vera)的患者进行了骨髓活检的形态计量分析,以揭示这些疾病在组织形态学特征上的显著差异。与无任何血液系统疾病患者的对照标本相比,巨核细胞增殖在PTH中最为显著,甚至超过伴有血小板增多症的P. vera(25例中有11例血小板计数大于600×10⁹/L)。此外,在P. vera中,巨核细胞大小范围广泛,包括微巨核细胞以及具有高度分叶核(四个核叶)的巨大形态,这使细胞呈现多形性外观。与正常骨髓相比,PTH中嗜中性粒细胞生成和红细胞生成的量没有显著增加,这与P. vera相反。关于网硬蛋白(嗜银)纤维的密度也可获得类似结果:对照标本和PTH中含量正常,而P. vera显示出最小到轻微的增加。最后,P. vera的骨髓完全没有可染色的铁,而在约三分之二无血液系统疾病的患者和PTH中检测到含铁血黄素沉积。这项形态计量学研究揭示的特征性差异可能会改善这些疾病中存在争议的组织学诊断。

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