Joundi A, Caulet T, Caulet S, Patey M, Pluot M, Blaise A M
Laboratoire Central d'Anatomie et de Cytologie Pathologiques, Faculté de Médecine, Reims.
Arch Anat Cytol Pathol. 1991;39(3):103-8.
This report describes three cases of acute malignant myelofibrosis characterized by pancytopenia, absence of splenomegaly, bone marrow fibrosis with an immature cell proliferation and rapidly fatal outcome. The authors investigated the origin of blast cells with the use of immunohistochemical markers on paraffin embedded material with anti-factor VIII, BNH9 and anti-lysozyme. They studied the expression of megakaryoblastic, erythroblastic and myeloblastic differentiation in these cells. They demonstrated the heterogeneity of blast cells which are capable of differentiating along the three hematologic cell lines. The morphometric study showed the mutilating or systematized character of myelofibrosis. The increase in reticulin fiber density compared with normal bone marrow was not significantly different from two other types of myelofibrosis. It would be interesting to correlate a quantitative parameter with the course of this disease in order to evaluate the prognosis and the treatment.
本报告描述了三例急性恶性骨髓纤维化病例，其特征为全血细胞减少、无脾肿大、骨髓纤维化伴幼稚细胞增殖且预后迅速死亡。作者使用抗因子VIII、BNH9和抗溶菌酶的免疫组化标记物，对石蜡包埋材料上的原始细胞来源进行了研究。他们研究了这些细胞中巨核母细胞、幼红细胞和髓母细胞分化的表达。他们证明了能够沿着三种血液学细胞系分化的原始细胞的异质性。形态计量学研究显示了骨髓纤维化的致残或系统化特征。与正常骨髓相比，网状纤维密度的增加与其他两种类型的骨髓纤维化无显著差异。将定量参数与该疾病的病程相关联以评估预后和治疗情况将是很有意思的。
