Wolf B C, Neiman R S
Blood. 1985 Apr;65(4):803-9.
We undertook a study of 35 cases of myelofibrosis with myeloid metaplasia to assess the histopathologic findings in the bone marrow of patients with this disorder, to ascertain if changes in morphology occurred with time, and to attempt to correlate marrow findings with splenic size. We reviewed 71 bone marrow biopsies and studied 13 splenectomy specimens. Sequential bone marrow biopsies were obtained in 21 cases over intervals ranging from two to ten years (mean, 4 1/2 years). We noted a patchy nature and variable degree of stromal proliferation in most marrow biopsies, and were unable to demonstrate a correlation between the extent of medullary fibrosis and duration of disease, splenic weight, or degree of splenic myeloid metaplasia. We were unable to document a progression of medullary fibrosis as a cause for increasing splenomegaly. However, the alteration in the marrow stroma in this disorder is responsible for the presence of distended marrow sinusoids with intravascular hematopoiesis, a phenomenon we observed in all cases. We believe that this morphological feature, not emphasized by previous investigators, is of significance in understanding the pathophysiology of myeloid metaplasia.
我们对35例伴有髓外化生的骨髓纤维化患者进行了一项研究，以评估该疾病患者骨髓的组织病理学表现，确定形态学变化是否随时间发生，并试图将骨髓 findings 与脾脏大小相关联。我们回顾了71份骨髓活检标本并研究了13份脾切除标本。在21例患者中，间隔两到十年（平均4.5年）获取了序贯骨髓活检标本。我们注意到大多数骨髓活检中存在斑片状性质和不同程度的基质增殖，并且无法证明髓样纤维化程度与疾病持续时间、脾脏重量或脾脏髓外化生程度之间存在相关性。我们无法证明髓样纤维化的进展是脾肿大增加的原因。然而，该疾病中骨髓基质的改变导致了扩张的骨髓血窦伴血管内造血的存在，这一现象我们在所有病例中均观察到。我们认为这一形态学特征（先前研究者未强调）对于理解髓外化生的病理生理学具有重要意义。
