Schultz William H, Ware Russell E
Duke University Medical Center, Durham, North Carolina 27710, USA.
Am J Hematol. 2003 Dec;74(4):249-53. doi: 10.1002/ajh.10427.
Malignancy in patients with sickle cell disease (SCD) has been previously reported, but the types of cancer and its incidence remain undefined. With the advent of hydroxyurea therapy, there is concern about increasing the cancer risk for patients with SCD. The International Association of Sickle Cell Nurses and Physician Assistants identified 52 cases of cancer (49 patients) among 16,613 patients with SCD followed at 52 institutions. The median age at malignancy diagnosis was 34 years (range, 14 months-62 years). Twenty-one cases (40%) occurred in pediatric patients, primarily leukemia (n = 7) or Wilms' tumor (n = 5), with 15 children surviving. Most adults had solid tumors, especially carcinomas, and only nine were known to be alive. Three patients received hydroxyurea before the diagnosis of malignancy. These data provide essential baseline information for the accurate interpretation of future reports of malignancy in patients with SCD, especially those receiving hydroxyurea therapy.
先前已有关于镰状细胞病（SCD）患者发生恶性肿瘤的报道，但癌症类型及其发病率仍不明确。随着羟基脲疗法的出现，人们担心这会增加SCD患者的癌症风险。国际镰状细胞病护士和医师助理协会在52家机构随访的16613例SCD患者中确定了52例癌症病例（49名患者）。恶性肿瘤诊断时的中位年龄为34岁（范围为14个月至62岁）。21例（40%）发生在儿科患者中，主要是白血病（n = 7）或肾母细胞瘤（n = 5），15名儿童存活。大多数成人患有实体瘤，尤其是 carcinomas，已知仅9人存活。3例患者在恶性肿瘤诊断前接受了羟基脲治疗。这些数据为准确解读SCD患者尤其是接受羟基脲治疗的患者未来的恶性肿瘤报告提供了重要的基线信息。
