Takahashi T, Kazama Y, Shimizu H, Yoshimoto M, Tsujisaki M, Aoki S, Imai K
Second Department of Internal Medicine, Tenshi Hospital, Sapporo, Hokkaido, Japan.
Int J Hematol. 2001 Jul;74(1):64-9. doi: 10.1007/BF02982551.
Patients with myelodysplastic syndrome (MDS) show a relatively high incidence of developing cancers. However, it is extremely rare that synchronous double cancers develop in an MDS patient. We report a case of MDS that progressed rapidly into erythroleukemia (M6 by French-American-British classification) complicated by gastric cancer and carcinoma of the papilla of Vater. A 66-year-old man was admitted because of pancytopenia with peripheral blasts. A diagnosis of MDS (with refractory anemia with excess of blasts in transformation [RAEB-T]) was made by bone marrow examination. Chromosome analysis revealed 46,XY. An early gastric cancer was also diagnosed by endoscopic examination. The peripheral blasts gradually proliferated and the disease progressed to M6. A chromosome abnormality 46,XY,del(1)(q42) was detected at the leukemic transformation. A CAG (low-dose cytarabine and aclarubicin in combination with granulocyte colony-stimulating factor) regimen was started as a remission-induction therapy. However, obstructive jaundice developed and a marked dilatation of bile ducts was observed by abdominal computed tomography (CT). A carcinoma of the papilla of Vater was detected by endoscopy. As remission was achieved and the pancytopenia improved, the patient subsequently underwent a surgical jejuno-choledochostomy to manage the jaundice. However, the leukemia relapsed thereafter and additional chromosome abnormalities including der(5)t(5;10)(p15:q11) were observed.
骨髓增生异常综合征（MDS）患者发生癌症的几率相对较高。然而，MDS患者同时发生双癌极为罕见。我们报告一例MDS迅速进展为红白血病（根据法美英分类为M6型）并合并胃癌和 Vater 乳头癌的病例。一名66岁男性因全血细胞减少伴外周血原始细胞增多入院。经骨髓检查诊断为MDS（转化中的原始细胞过多的难治性贫血[RAEB-T]）。染色体分析显示为46,XY。内镜检查还诊断出早期胃癌。外周血原始细胞逐渐增殖，疾病进展为M6型。白血病转化时检测到染色体异常46,XY,del(1)(q42)。开始采用CAG（小剂量阿糖胞苷、阿柔比星联合粒细胞集落刺激因子）方案进行缓解诱导治疗。然而，出现了梗阻性黄疸，腹部计算机断层扫描（CT）观察到胆管明显扩张。内镜检查发现 Vater 乳头癌。随着病情缓解和全血细胞减少改善，患者随后接受了手术空肠胆管吻合术以治疗黄疸。然而，此后白血病复发，观察到包括der(5)t(5;10)(p15:q11)在内的其他染色体异常。
