原发性骨髓硬化症伴肝脾髓样化生的临床分析

Primary myelofibrosis with myeloed metaplasia of spleen and liver: areport of two cases and review of the literature

目的 探讨原发性骨髓硬化症伴肝脾髓样化生的临床病理特点、诊断及鉴别诊断。方法 光镜下进行组织学观察、末梢血及骨髓穿刺涂片检查 ,并结合文献进行分析。结果 肝脾镜下均出现髓外造血灶 ,灶内细胞成分由有核红细胞、巨核细胞和粒细胞三者以不同比例构成。末梢血涂片 :背景见大量血小板 ,并可见幼粒、幼红细胞。骨髓穿刺 :细胞成分稀少的纤维化骨髓组织中含有多数核异形巨核细胞。结论 原发性骨髓硬化症伴肝脾髓样化生是一种慢性进行性疾病 ,病因不清 。

Objective To study the clinicopathology,diagnosis and differential diagnosis of Primary myelofibrosis with myeloid metaplasia of live and spleed.Methods Light microscopy was used for histological investigation, peripheral smear and bone marrow puncture wased examined with review of the literature.Results Under the Light microscopy, liver and spleen appear the blood cooking stove outside bone marrow, there are three cell component in the blood cooking stove, it is nucleated erythrocye and promegakaryocyte and metamyelocyte. peripheral smear: The background can observed a lot of blood platelets, and normoblast and metamyelocyte. Bone marrow puncture: there was massive fiber hyperplasia with a few of cell component on the bone marrow puncture and there was more promegakaryocyte with nuclear atypta.Conclusion Primary myelofibrosis with myeloid metaplasia is a chronic proceeding disease. the etiopathology of primary myelofibrosis with myeloid metaplasia is not clear.Recognizing this a seldom disease is important to avoid confusion with other similar lesions.