Alter B P
Division of Pediatric Hematology/Oncology, University of Texas Medical Branch, Galveston 77555-0361, USA.
Am J Hematol. 1996 Oct;53(2):99-110. doi: 10.1002/(SICI)1096-8652(199610)53:2<99::AID-AJH7>3.0.CO;2-Z.
Patients with Fanconi's anemia (FA) are at a high risk for development of malignancies. It is well-known that leukemia occurs in approximately 10% of cases, with increasing risk with age. Less commonly recognized is the risk for myelodysplastic syndromes (approximately 5%); the relationship between myelodysplasia and evolution to leukemia remains speculative. What also needs to be emphasized is that older patients have an ever-increasing risk for development of solid tumors, with at least 5% reported to have liver tumors (male:female ratio, 2:1) and an equal number of other cancers (female:male ratio, 3:1, even after exclusion of gynecologic malignancies). Hematologists have tended to focus on aplastic anemia and leukemia. As FA patients live longer, more of the other malignancies will occur, perhaps related to cord blood or bone marrow transplant, or treatment with cytokines. This review identifies the types of tumors for which patients with Fanconi's anemia are at risk.
范可尼贫血(FA)患者发生恶性肿瘤的风险很高。众所周知,约10%的病例会发生白血病,且风险随年龄增长而增加。骨髓增生异常综合征的风险(约5%)则较少被认识到;骨髓增生异常与白血病演变之间的关系仍具有推测性。还需要强调的是,老年患者发生实体瘤的风险不断增加,据报道至少5%的患者患有肝肿瘤(男女比例为2:1),其他癌症的发生率相同(女性与男性比例为3:1,即使排除妇科恶性肿瘤)。血液学家往往将重点放在再生障碍性贫血和白血病上。随着FA患者寿命延长,可能与脐血或骨髓移植或细胞因子治疗有关,其他更多的恶性肿瘤将会出现。本综述确定了范可尼贫血患者面临风险的肿瘤类型。