Strebel U, Schaffner A, Fehr J
Schweiz Med Wochenschr. 1983 Jun 11;113(23):844-50.
Two cases of acute myelofibrosis are described which exemplify diagnostic and therapeutic possibilities in this clinical and morphologic entity. Delimiting criteria against other diseases with bone marrow fibrosis are also presented. This malignant myeloproliferative disorder is characterized by acute onset, minimal or absent splenomegaly, blood cytopenias with a highly variable percentage of blast cells in circulating blood, often unimpressive tear-drop poikilocytosis, intense marrow fibrosis, and a rapid clinical course. In one case, typical proliferation of all three hematopoietic cell lines was lacking and proliferating blast cells could be classified as myeloblasts/promyelocytes histochemically. In the other case, a single course of intensive combination chemotherapy resulted in marked dissolution of the marrow fibrosis and prolonged survival in partial remission.
本文描述了两例急性骨髓纤维化病例，例证了该临床和形态学实体中的诊断和治疗可能性。还介绍了与其他伴有骨髓纤维化疾病的鉴别标准。这种恶性骨髓增殖性疾病的特点是起病急，脾肿大轻微或无，血细胞减少，循环血液中原始细胞百分比变化很大，泪滴状异形红细胞增多症往往不明显，骨髓纤维化严重，临床病程进展迅速。其中一例缺乏所有三种造血细胞系的典型增殖，增殖的原始细胞在组织化学上可归类为原粒细胞/早幼粒细胞。另一例患者接受一个疗程的强化联合化疗后，骨髓纤维化明显消退，部分缓解期生存期延长。
