一例伴有Ph染色体的获得性无巨核细胞性血小板减少性紫癜

报道１例伴有Ｐｈ染色体的获得性无巨核细胞性血小板减少性紫癜，患者最初有鼻衄、牙龈出血和紫癜，血小板严重减少，骨髓巨核细胞缺如，而其他造血细胞正常。经泼尼松治疗无效，两年后演变为全血细胞减少并死于颅内出血。ＣＦＵ－ＧＭ检测显示集落生长甚少。骨髓细胞染色体分析揭示典型的Ｐｈ易位，但分子学研究未见ｂｃｒ重排。

A PATIENT WITH ACQUIRED AMEGAKARYOCYTICTHROMBOCYTOPENIC PURPURA AND A PHCHROMOSOME

patient with acquired amegakaryocytic thrombocy-topenic purpura and a Ph chromosome was first report-ed. He had severe thromtheytopenia resulting in epis-taxis, gingival bleeding and ecchymoses, while otherhematological values were within nomal range. Bonemarrow aspiration showed no megakaryocyte with a nor-mal appearance of erythroblastic and granulopoietic se-ries. He failed to respond to prednisone and underwent aprogress from isolated thrombocytopenia to full pancy-topenia. At last, he died of spontanious intracranialhemorrhage. An in vitro culture for granulocyte-maerophage precusors showed very few colonies. Kary-otypic analysis revealed a standard Ph translocation,t ( 9;22) (q34 ;q11). Southern blot analysis with a 3' bcr-HE probe didni't detected a rearrangement within the bcrDNA sequence.