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骨髓增殖性疾病中的骨髓淋巴小结:与特发性骨髓纤维化的非骨髓硬化期的关联及免疫学意义

Bone marrow lymphoid nodules in myeloproliferative disorders: association with the nonmyelosclerotic phases of idiopathic myelofibrosis and immunological significance.

作者信息

Cervantes F, Pereira A, Marti J M, Feliu E, Rozman C

机构信息

Postgraduate School of Haematology Farreras Valenti, Hospital Clínico y Provincial, University of Barcelona, Spain.

出版信息

Br J Haematol. 1988 Nov;70(3):279-82. doi: 10.1111/j.1365-2141.1988.tb02482.x.

Abstract

The presence of lymphoid nodules in bone marrow biopsy was investigated at diagnosis in 200 patients with chronic myeloproliferative disorders (MPD). Twelve out of 51 patients with idiopathic myelofibrosis (IM) showed such a feature (23.5%), versus two out of 100 with Ph1-positive chronic myeloid leukaemia, two of 32 with polycythaemia vera, and one of 17 with essential thrombocythaemia, the difference between IM and the remaining MPD being statistically significant (P less than 0.0001). When IM patients were compared for their initial characteristics according to the presence or not of bone marrow lymphoid nodules, patients with such a histological finding showed significantly lower values for either WBC counts, number of primitive cells in the blood, and serum lactic dehydrogenase levels. Moreover, it was observed that virtually all patients with lymphoid nodules were in the nonmyelosclerotic phases of IM. Finally, among the 14 of 32 IM patients (44%) investigated for circulating immune complexes who gave a positive test, a significant association between this immunological abnormality and bone marrow lymphoid nodules was found. The above results reinforce the immunological significance of the finding of bone marrow lymphoid nodules in IM and give support to the hypothesis of an immune component in the pathogenesis of the disorder.

摘要

在200例慢性骨髓增殖性疾病(MPD)患者诊断时,对骨髓活检中淋巴小结的存在情况进行了研究。51例原发性骨髓纤维化(IM)患者中有12例表现出这一特征(23.5%),而100例Ph1阳性慢性髓性白血病患者中有2例,32例真性红细胞增多症患者中有2例,17例原发性血小板增多症患者中有1例出现该特征,IM与其余MPD之间的差异具有统计学意义(P小于0.0001)。根据骨髓淋巴小结的有无对IM患者的初始特征进行比较时,有这种组织学表现的患者白细胞计数、血液中原始细胞数量和血清乳酸脱氢酶水平的值均显著较低。此外,观察到几乎所有有淋巴小结的患者都处于IM的非骨髓硬化阶段。最后,在32例接受循环免疫复合物检测的IM患者中,有14例(44%)检测呈阳性,发现这种免疫异常与骨髓淋巴小结之间存在显著关联。上述结果强化了IM中骨髓淋巴小结发现的免疫学意义,并支持了该疾病发病机制中存在免疫成分的假说。

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