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原发性骨髓纤维化是一种原因不明的骨髓纤维组织增生性疾病,属骨髓增生综合征范畴.我院自1978年以来诊断8例,现分析如下.临床资料一、性别、年龄男5例、女3例,平均38岁(20～54岁),病程半年至8年.二、症状体征均有贫血.皮肤牙龈出血3例,月经过多1例,脾肿大6例,肝肿大5例,淋巴结肿大2例.三、实验室检查 8例血红蛋白为25～100g/L,3例末梢血可见幼红细胞,6例有畸形红细胞.网织红细胞数3例升高、血沉5例增快.5例白细胞介于10.0～25.0×10~9/L,出现幼粒细胞4例.血小板数2例低于 10~(11)/L,3例高于400×10~9/L。骨髓象:干抽3例,仅1例骨髓增生低下,7例增生活跃.骨髓活检,8例均呈不同程度的纤维组织增生.1例作脾穿刺有幼粒、幼红细胞。乙肝表面抗原阳性者2例.
Primary myelofibrosis is an unexplained myelofibrosis, a category of myeloproliferative syndrome in our hospital since 1978, the diagnosis of 8 cases, are as follows.Clinical data A sex, age 5 male , 3 females, average 38 years old (20 to 54 years old), duration of six months to 8 years. Second, the symptoms and signs are anemic .Grain bleeding in the skin in 3 cases, 1 case of excessive menstruation, splenomegaly in 6 cases, hepatomegaly 5 cases, 2 cases of lymphadenopathy .In three, laboratory examination of 8 cases of hemoglobin 25 ~ 100g / L, 3 cases of peripheral blood visible red blood cells, 6 cases of deformity red blood cells.Reticangular cell count increased in 3 cases, 5 cases of erythrocyte sedimentation rate In 5 cases of leucocytes ranging from 10.0 to 25.0 × 10 ~ 9 / L, there were 4 cases of promyelocytic cells.The number of platelets in 2 cases was lower than 10 ~ (11) / L, and in 3 cases more than 400 × 10 ~ 9 / L . Bone marrow: dry pumping in 3 cases, only 1 case of low bone marrow hyperplasia, hyperplasia in 7 cases active .Bone marrow biopsy, 8 cases showed varying degrees of fibrous tissue hyperplasia .1 cases of splenic punctate with immature erythrocytes. Hepatitis B surface antigen positive in 2 cases.