Fujiwara H, Takahashi N, Tada J, Higuchi T, Harada H, Mori H, Niikura H, Omine M, Fujita K
Department of Internal Medicine, Showa University Fujigaoka Hospital.
Rinsho Ketsueki. 1997 Aug;38(8):663-68.
A 66-year-old woman complained of chest discomfort in January 1995. In March the accelerated phase of chronic myeloid leukemia (CML) was diagnosed. Chromosomal analysis demonstrated negative Ph and positive t(9;16) (q34;p11) with positive major BCR/ABL chimeric mRNA. Administration of hydroxycarbamide was initiated, but in May she developed high fever and severe left hypochondralgia. Her WBC was 62,100/microliter (blast 64%), and LDH was 3,590 IU/l. Bone marrow examination showed 78.6% blasts, with a nucleated cell count of 74 x 10(3)/microliter. Blasts were negative for esterase stain and partially positive for both peroxidase stain and PAS reaction. Surface marker analysis revealed that blasts were positive for CD13, CD19, CD33, CD34, and HLA-DR. A diagnosis of blast crisis was made and she was treated with the VDS-CP regimen with heparin for DIC. After temporary improvement her disease recurred rapidly with severe DIC. Treatment with low molecular weight heparin and fresh frozen plasma failed to control DIC and she died of subarachnoid hemorrhage on the 48th hospital day. This is the first veprted of case Ph-negative, M-BCR/ABL-positive CML with t(9;16) accompanied by severe DIC.
一名66岁女性于1995年1月主诉胸部不适。3月诊断为慢性髓性白血病（CML）加速期。染色体分析显示Ph阴性，t(9;16) (q34;p11)阳性，主要BCR/ABL嵌合mRNA阳性。开始给予羟基脲治疗，但5月她出现高热和严重左季肋部疼痛。她的白细胞计数为62,100/微升（原始细胞64%），乳酸脱氢酶为3,590 IU/l。骨髓检查显示原始细胞占78.6%，有核细胞计数为74×10(3)/微升。原始细胞酯酶染色阴性，过氧化物酶染色和PAS反应部分阳性。表面标志物分析显示原始细胞CD13、CD19、CD33、CD34和HLA-DR阳性。诊断为原始细胞危象，她接受了VDS-CP方案联合肝素治疗弥散性血管内凝血（DIC）。病情暂时改善后，疾病迅速复发并伴有严重DIC。低分子量肝素和新鲜冰冻血浆治疗未能控制DIC，她在住院第48天死于蛛网膜下腔出血。这是首例报道的伴有严重DIC的Ph阴性、M-BCR/ABL阳性且有t(9;16)的CML病例。
