Concern has emerged about the prevalence of second cancers among patients with hairy cell leukemia (HCL) treated with purine analogs. We investigated 513 patients with HCL treated with cladribine over the last 30 years at 18 Italian centers and calculated their standardized incidence ratios (SIRs). We identified 24 patients with a second cancer diagnosed at a median time from treatment with cladribine of 59.9 months (range: 9.2–169.7 months). All patients with solid neoplasms presented with a limited-stage disease, except four cases of locally advanced cancer; multiple myeloma patients had a smoldering disease, while lymphoma patients had stage Ie and stage IV diseases. Response to therapy was complete in 19 cases; 1 patient is still receiving treatment for a relapsing bladder disease, while 2 patients progressed during treatment and died. These two patients died from unrelated causes: one from infection and one due to surgery complications. The median OS from HCL was 98.5 months (range: 38.4–409.2 months), while the median OS from second cancer was 27.6 months (range: 1–117.8 months). The SIR was 0.86 (95% CI: 0.54–1.30) for males and 1.13 (95% CI: 0.36–2.73) for females: no statistically significant differences were highlighted. We were not able to demonstrate an excess of second cancer or a significant association with the specific studied neoplasm.
关于使用嘌呤类似物治疗的毛细胞白血病（HCL）患者中第二癌症的患病率问题已引起关注。我们调查了意大利18个中心在过去30年间接受克拉屈滨治疗的513例HCL患者，并计算了其标准化发病率比（SIR）。共发现24例患者在克拉屈滨治疗后中位时间59.9个月（范围：9.2-169.7个月）被诊断为第二癌症。除4例局部晚期癌症外，所有实体肿瘤患者均表现为局限期病变；多发性骨髓瘤患者为隐匿性疾病，而淋巴瘤患者分别为Ie期和IV期病变。19例患者对治疗达到完全缓解；1例患者因复发性膀胱疾病仍在接受治疗，2例患者在治疗期间病情进展并死亡。这两例患者的死亡原因与癌症无关：一例死于感染，另一例死于手术并发症。从HCL诊断起的中位总生存期为98.5个月（范围：38.4-409.2个月），而从第二癌症诊断起的中位总生存期为27.6个月（范围：1-117.8个月）。男性SIR为0.86（95% CI：0.54-1.30），女性为1.13（95% CI：0.36-2.73）：未发现具有统计学显著性的差异。我们未能证实第二癌症发生率异常增高或与特定研究肿瘤存在显著关联。
肿瘤(癌症)患者之家