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慢性骨髓增殖性疾病中的巨核细胞生成。一项形态学评估,特别关注原发性血小板增多症。

Megakaryopoiesis in chronic myeloproliferative diseases. A morphometric evaluation with special emphasis on primary thrombocythemia.

作者信息

Thiele J, Funke S, Holgado S, Choritz H, Georgii A

出版信息

Anal Quant Cytol. 1984 Sep;6(3):155-67.

PMID:6594964
Abstract

Morphometry was employed on different entities of chronic myeloproliferative diseases (CMPD) and reactive lesions in addition to normal control specimens. The entities studied included: (1) inflammatory reactions of the bone marrow (so-called myelitis in chronic rheumatoid arthritis), (2) chronic granulocytic leukemia (CGL), (3) agnogenic myeloid metaplasia in an early hypercellular stage (so-called chronic megakaryocytic-granulocytic myelosis, CMGM), (4) agnogenic myeloid metaplasia in an advanced fibrosclerotic stage or osteomyelofibrosis/sclerosis (MF/OMS), (5) polycythemia vera (P. vera), (6) reactive thrombocytosis (TH, as a sequel of miscellaneous conditions) and (7) primary (idiopathic, essential) thrombocythemia (PTH). Evaluation was done on plastic-embedded semithin sections with a constant thickness of 3 micron in approximately 20 cases of each group of CMPD. The following parameters were determined: (1) density distributions of the megakaryocyte and non-megakaryocyte compartments, (2) arrangement of megakaryopoiesis in the bone marrow space (i.e., inhomogeneity or clustering) and (3) the fine structure of megakaryocytes in PTH, with a quantitative analysis of the nuclear morphology by circular deviation and contour factors. The megakaryocyte morphology was closely related to a facultative or obligatory increase of the platelet count in these various entities of CMPD and was separable into two major categories: (1) controls, CGL and myelitis versus (2) CMGM, MF/OMS, P. vera, TH and PTH. These two categories were distinguishable by the prominence of megakaryopoiesis in the bone marrow as well as the elevated platelet counts in the periphery. Moreover, in comparison with CMGM and MF/OMS, PTH was characterized by an apparently normal maturation and a conspicuous polyploidization of megakaryocytes according to the nuclear morphology, which was similar to that of P. vera. Our results suggest that PTH presents a monolinear growth of the megakaryopoiesis in the same way as CGL exhibits a monolinear proliferation of the neutrophilic granulopoiesis. This is in contrast to the mixed cellularity of both the megakaryocyte and granulocyte lineage in CMGM and MF/OMS.

摘要

除正常对照标本外,对慢性骨髓增殖性疾病(CMPD)的不同类型以及反应性病变进行了形态计量学研究。研究的类型包括:(1)骨髓的炎症反应(慢性类风湿性关节炎中所谓的骨髓炎),(2)慢性粒细胞白血病(CGL),(3)早期细胞增多阶段的特发性骨髓化生(所谓的慢性巨核细胞 - 粒细胞骨髓增生症,CMGM),(4)晚期纤维硬化阶段的特发性骨髓化生或骨髓纤维化/硬化症(MF/OMS),(5)真性红细胞增多症(P. vera),(6)反应性血小板增多症(TH,作为各种病症的后遗症)和(7)原发性(特发性、本质性)血小板增多症(PTH)。对每组CMPD中约20例病例的厚度恒定为3微米的塑料包埋半薄切片进行评估。测定了以下参数:(1)巨核细胞和非巨核细胞区室的密度分布,(2)骨髓空间中巨核细胞生成的排列(即不均匀性或聚集性)以及(3)PTH中巨核细胞的精细结构,并通过圆形偏差和轮廓因子对核形态进行定量分析。在这些不同类型的CMPD中,巨核细胞形态与血小板计数的兼性或强制性增加密切相关,可分为两大类:(1)对照、CGL和骨髓炎与(2)CMGM、MF/OMS、P. vera、TH和PTH。这两类可通过骨髓中巨核细胞生成的突出程度以及外周血小板计数升高来区分。此外,与CMGM和MF/OMS相比,根据核形态,PTH的特征是巨核细胞明显正常成熟和显著多倍体化,这与P. vera相似。我们的结果表明,PTH呈现出巨核细胞生成的单线性生长,就像CGL表现出嗜中性粒细胞生成的单线性增殖一样。这与CMGM和MF/OMS中巨核细胞系和粒细胞系的混合细胞性形成对比。

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