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骨髓纤维化(MF)通常见于中年人及老年人,平均年龄为60岁。其特征为周围血象呈幼粒幼红细胞性贫血,脾肿大,髓外造血及骨髓纤维化。本病病程一般每况愈下,确诊后的中位存活期为1-5年。年轻成年人的MF自然病程是否与老年人的相同则不明。作者报道3例年轻成年人的MF,骨髓穿刺均呈干抽,经骨髓活检(细胞成分正常至减少),网硬蛋白染色(例1、2呈中度纤维化,例3轻度纤维化)及肝活检(均发现髓外造血的证据)而确诊。例2,女,23岁。体检时发现脾肋下7cm,肝肋下3cm。血红蛋白11.4g/dl,红细胞指数正常,网织红细胞2%。白细胞总数6×10~9/l,其中多形核50%、
Myelofibrosis (MF) is usually found in middle-aged and elderly people, with an average age of 60 years. It is characterized by peripheral blood showed promyelocytic anemia, splenomegaly, extramedullary hematopoiesis and myelofibrosis. The course of the disease generally deteriorating, the median survival after diagnosis of 1-5 years. It is not yet clear whether the natural history of MF in young adults is the same as in older adults. The authors report that MF and bone marrow aspirates from 3 young adult patients showed dry pumping, bone marrow biopsy (normal to reduced cellular components), nether protein staining (moderate fibrosis in 1 and 2, mild fibrosis in 3) And liver biopsy (evidence of extramedullary hematopoiesis) were confirmed. Example 2, female, 23 years old. Physical examination found spleen ribs 7cm, liver ribs 3cm. Hemoglobin 11.4g / dl, normal red blood cell index, reticulocyte 2%. The total number of leukocytes 6 × 10 ~ 9 / l, of which polymorphonuclear 50%