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[原发性骨髓纤维化前期骨髓纤维化的发展。对38例患者进行序贯骨髓活检以研究其进展情况]

[The development of myelofibrosis in prefibrotic cIMF. An investigation of the progression by sequential bone marrow biopsies in 38 patients].

作者信息

Buhr T, Länger F, Kreft A, Büsche G, Choritz H, Kreipe H

机构信息

Institut für Pathologie, Medizinische Hochschule, Carl-Neuberg-Strasse 1, 30625 Hannover, Germany.

出版信息

Pathologe. 2002 Nov;23(6):433-7. doi: 10.1007/s00292-002-0582-9. Epub 2002 Oct 19.

DOI:10.1007/s00292-002-0582-9
PMID:12436296
Abstract

Overt myelofibrosis in bone marrow biopsies as a diagnostic postulate in cIMF has been discarded only recently by the WHO. Therefore, only a few studies have been performed on the evolution of myelofibrosis in "prefibrotic" cIMF by means of sequential bone marrow biopsies. We carried out this study on 38 patients, split up into two groups, A and B according to treatment modalities, to evaluate the dynamics and frequency of myelofibrosis in both groups. Our results indicate a step-wise development of myelofibrosis from a "prefibrotic" to a "classical" cIMF, as 75-80% of the respective patients in both groups progressed to myelofibrosis. However, this evolution seems to be heterogeneous and unpredictable in individual patients, since myelofibrosis could be seen as early as less than 2 years after diagnosis in 12/38 (31.6%) patients, whereas 3 patients remained "prefibrotic" even after up to 6 years of follow-up.

摘要

骨髓活检中的明显骨髓纤维化作为慢性原发性骨髓纤维化(cIMF)的一种诊断假设,直到最近才被世界卫生组织摒弃。因此,仅有少数研究通过序贯骨髓活检对“纤维化前期”cIMF中骨髓纤维化的演变进行了探讨。我们对38例患者进行了此项研究,根据治疗方式将其分为A组和B组,以评估两组中骨髓纤维化的动态变化及发生率。我们的结果表明,骨髓纤维化从“纤维化前期”到“经典”cIMF呈逐步发展,因为两组中分别有75 - 80%的患者进展为骨髓纤维化。然而,这种演变在个体患者中似乎具有异质性且不可预测,因为在12/38(31.6%)的患者中,骨髓纤维化在诊断后不到2年时就已出现,而即使经过长达6年的随访,仍有3例患者处于“纤维化前期”。

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