【摘要】 <正>骨髓增殖性疾病(myeloproliferative diseases,MPD)是一组多能造血干细胞的克隆性疾病,表现为一系或多系骨髓细胞的异常增殖。MPD在欧美国家中并不少见,总的发生率为6~10/(10万人口·年),好发于50岁以上的中老年人,男性多见〔1〕,而在我国尚无可靠的流行病学调查。随着JAK2V617F等基因突变的发现,MPD的概念演变成了骨髓增殖性肿瘤(myeloproliferative neoplasms,MPN)〔2〕。MPN是一种非遗传性疾病,多以散发病例存在,但亦有家族聚集性,称为家族性MPN,国外有相关的文献报道,而国内相关文献报道少见。我治疗中心有同胞姐弟2例患此病,现报告如下。更多还原

【Abstract】 Two cases of fellow siblings with myeloproliferative neoplasms were admitted.The sister was diagnosed with overlap syndrome(essential thrombocythemia and myelofibrosis)and the brother was diagnosed with myelofibrosis.Now we will review the incidence,pathogenesis,clinical features and prognosis of familial myeloproliferative neoplasms.更多还原