原发性血小板增多症是骨髓增殖异常综合症(Myelodysplastis Syndrome)之一,也有人认为是一种独立性疾病.1920年DiGulielmo首次报告,1934年Epstein鉴于本病常有出血症状,首称为出血性血小板增多症(Hemorrhagie throbocythemia)极为少见.Gunz认为1960年以前全世界仅有50例符合本病.国内1959年孟宪镛开始报告一例,至今仅有13例报告. Essential thrombocythemia is one of the Myelodysplastic Syndromes, some considered as an independent disease. DiGulielmo was first reported in 1920, Epstein’s disease in 1934 due to the presence of bleeding symptoms, the first known as bleeding Hemorrhagie throbocythemia is extremely rare, and Gunz said only 50 of the world’s patients were eligible for the disease before 1960. In 1959, Meng Xian-tao began reporting one case, and so far only 13 cases have been reported.