Thiele J, Kvasnicka H M, Schmitt-Graeff A, Diehl V
Institute of Pathology, University of Cologne, Cologne, Germany.
Histopathology. 2003 Nov;43(5):470-9. doi: 10.1046/j.1365-2559.2003.01732.x.
To analyse systematically therapy-induced lesions of haematopoiesis in chronic idiopathic myelofibrosis (IMF).
A total of 759 sequential bone marrow (BM) biopsies (median interval 32 months) were performed in 261 patients with IMF. Besides a control group (symptomatic treatment), monotherapies included busulfan, hydroxyurea and interferon. In all therapy groups hypoplasia of varying degree was a frequent finding and often accompanied by a patchy distribution of haematopoiesis. Most conspicuous was gelatinous oedema showing a tendency to develop discrete reticulin fibrosis (scleroedema). Minimal to moderate maturation defects of megakaryopoiesis and erythroid precursors occurred, but overt myelodysplastic features were most prominent following hydroxyurea and busulfan therapy. Acceleration and blastic crisis were characterized by the appearance of immature and CD34+ progenitor cells. Concerning the dynamics of fibrosis, no differences were observed between controls and the various therapy groups. In 143 patients (55%) without or with little reticulin at onset, an increase in myelofibrosis was detectable that progressed to overt collagen fibrosis.
Therapy-related bone marrow lesions in IMF comprise a strikingly variable spectrum that may include aplasia with scleroedema and a patchy distribution of myelodysplastic haematopoiesis associated with progressive myelofibrosis.
系统分析慢性特发性骨髓纤维化（IMF）中治疗引起的造血损伤。
对261例IMF患者进行了总共759次连续骨髓活检（中位间隔32个月）。除了对照组（对症治疗），单一疗法包括白消安、羟基脲和干扰素。在所有治疗组中，不同程度的造血功能低下是常见表现，且常伴有造血的斑片状分布。最明显的是胶样水肿，有发展为离散性网状纤维纤维化（硬化性水肿）的趋势。巨核细胞生成和红系前体细胞出现轻度至中度成熟缺陷，但羟基脲和白消安治疗后明显的骨髓增生异常特征最为突出。加速期和原始细胞危象的特征是出现未成熟和CD34+祖细胞。关于纤维化的动态变化，对照组与各治疗组之间未观察到差异。在143例（55%）起病时无或仅有少量网状纤维的患者中，可检测到骨髓纤维化增加，并进展为明显的胶原纤维化。
IMF中与治疗相关的骨髓病变包括一系列显著不同的表现，可能包括伴有硬化性水肿的再生障碍以及与进行性骨髓纤维化相关的斑片状骨髓增生异常造血。
