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作者长期随访288例真性红细胞增多症(PV)患者,发现33例(11.5%)恶性变,这些病人初始符合PV诊断标准,主要采取~(32)P放疗、放血术及化疗。16/33例转化为伴原始细胞增多的难治性贫血(RAEB),其特征为:细胞数减少且严重畸变、三系减少不平行、外周血原始细胞缺如或中等度升高、骨髓原始细胞平均15%。其中7例最后转为白血病(3例M_2,2例M_6)并很快死亡(10天至2月)。17/33例未经过骨髓增生异常(MDS)而转化为急性非淋巴细胞性白血病(ANLL),其中M_14例、M_26例、M_51例、M_62例。除2例分别生存8个月和18个月外,其余均在诊断后3月内死亡。通用的化疗方案对这样的病例效果差。
Long-term follow-up of 288 patients with polycythemia vera (PV), 33 patients (11.5%) were found to have malignant transformation. These patients initially met the diagnostic criteria of PV, mainly taking ~ (32) P radiotherapy, radiotherapy and chemotherapy. 16/33 patients converted to refractory anemia (RAEB) with an increase of blasts, characterized by reduced and severely degenerated cells, non-parallel reduction of three lines, absence or moderate elevation of peripheral blood blasts, The average cell 15%. Of these, 7 were eventually converted to leukemia (3 M_2, 2 M_6) and died quickly (10 days to 2 months). 17/33 cases were transformed into acute non-lymphocytic leukemia (ANLL) without myelodysplastic syndrome (MDS), including M_14 cases, M_26 cases, M_51 cases and M_62 cases. Except 2 patients surviving for 8 months and 18 months respectively, the rest died within 3 months after diagnosis. Common chemotherapy regimens are ineffective in such cases.