Kuno Y, Takeo T, Kawashima K
Department of Internal Medicine, Yokkaichi Municipal Hospital.
Rinsho Ketsueki. 1997 Sep;38(9):782-7.
We reported a 72-year-old female patient who developed acute leukemia following a long course of polycythemia vera (PV). For 12 years she had been treated with phlebotomy, nimustine, busulfan, hydroxyurea and irradiation on splenomegaly. In November 1995, her peripheral blood smear showed blast of 30%. Bone marrow blasts were microscopically as well as electromicroscopically peroxidase-negative and CD7 and HLA-DR positive. Six months later, the blasts were positive for CD7, CD34 and HLA-DR. On the basis of morphologic, biochemical and immunophenotypic features, the patient was diagnosed acute leukemia, probably arising at a primitive multipotential stem cell level. She failed to respond to the various combination therapy including prednisolone, vincristine, cytarabine, daunorubicin and etoposide. The stem-cell-leukemia transformation in PV occurs rarely and may be refractory to chemotherapy.
我们报告了一名72岁女性患者,她在长期患真性红细胞增多症(PV)后发展为急性白血病。12年来,她一直接受放血治疗、尼莫司汀、白消安、羟基脲治疗以及针对脾肿大的放射治疗。1995年11月,她的外周血涂片显示原始细胞占30%。骨髓原始细胞在显微镜下以及电镜下过氧化物酶阴性,CD7和HLA-DR阳性。6个月后,原始细胞CD7、CD34和HLA-DR呈阳性。根据形态学、生化和免疫表型特征,该患者被诊断为急性白血病,可能起源于原始多能干细胞水平。她对包括泼尼松龙、长春新碱、阿糖胞苷、柔红霉素和依托泊苷在内的各种联合治疗均无反应。PV中的干细胞白血病转化很少发生,且可能对化疗耐药。