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慢性粒细胞白血病中的骨髓纤维化

Myelofibrosis in chronic granulocytic leukaemia.

作者信息

Clough V, Geary C G, Hashmi K, Davson J, Knowlson T

出版信息

Br J Haematol. 1979 Aug;42(4):515-26. doi: 10.1111/j.1365-2141.1979.tb01164.x.

Abstract

Serial trephine biopsies were performed in 45 cases of chronic granulocytic leukaemia (CGL) in order to determine the frequency and significance of secondary myelofibrosis in the evolution of the disease. Histological changes were graded 1-5b, ranging from no increase in reticulin to dense osteomyelosclerosis. Many cases showed a progressive increase from Grade 1 to Grade 3, and accelerated disease, or blast crisis, often supervened when Grade 3 changes were present. However, a significant number of cases showed Grade 4 and 5 changes, which were indistinguishable histologically from those found in agnogenic myeloid metaplasia (AMM) (idiopathic myelofibrosis), at the time of diagnosis. These patients did not always show a rapidly fatal course and may be considered as an example of 'transitional myeloproliferative disorder', with features intermediate between CGL and AMM.

摘要

对45例慢性粒细胞白血病(CGL)患者进行了连续环钻活检,以确定继发性骨髓纤维化在疾病进展过程中的发生率及意义。组织学改变分为1 - 5b级,从网状纤维无增加到致密性骨骨髓硬化不等。许多病例显示从1级逐渐进展到3级,当出现3级改变时,常继发加速期疾病或急变期。然而,相当数量的病例在诊断时显示为4级和5级改变,组织学上与原发性骨髓化生(AMM)(特发性骨髓纤维化)难以区分。这些患者并非总是表现出快速致命的病程,可被视为“过渡性骨髓增殖性疾病”的一个例子,其特征介于CGL和AMM之间。

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