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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。
One case of acquired megakaryocytic thrombocytopenic purpura accompanied by Ph chromosome was reported. The patient initially had epistaxis, gingival hemorrhage and purpura, severe reduction of platelets, absence of bone marrow megakaryocytes, and normalization of other hematopoietic cells. After prednisone treatment fails, two years later evolved into pancytopenia and died of intracranial hemorrhage. CFU-GM assay showed few colonies. Chromosomal analysis of bone marrow cells reveals typical Ph translocations, but no bcr rearrangements have been found in molecular studies.