Karimi Mehran, Giti Rahil, Haghpanah Sezaneh, Azarkeivan Azita, Hoofar Hamid, Eslami Masoomeh
Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Pediatr Blood Cancer. 2009 Dec;53(6):1064-7. doi: 10.1002/pbc.22144.
Beta thalassemia is one of the most common genetic disorders in the world. The aim of this study was to determine the frequency, characteristics, and pattern of malignancies in patients with beta thalassemia major (BTM) and beta thalassemia intermedia (BTI) in Iran.
We conducted a multicenter study via a retrospective chart review of patients with BTM and BTI between 2002 and 2007 in four thalassemia centers in Iran. A total of 4,630 records of patients with thalassemia were evaluated. Statistical analyses were done with SPSS software v. 15. P-values <0.05 were considered significant.
We detected 11 patients with beta thalassemia who also had malignant disease. Five patients (45.4%) were diagnosed with lymphoma and five with leukemia. The proportion of patients with cancer was higher in those with BTI. Cancer was diagnosed in patients with thalassemia aged 0-39 years, but not in any of the older patients. In patients with thalassemia overall and in patients with BTI, the highest age-specific rate of cancer incidence was seen in children <10 years old, whereas in the BTM group the highest incidence was observed in patients 20-29 years old. There were no statistically significant differences between patients with BTM and BTI and cancer regarding age, sex, splenectomy, hemoglobin, ferritin level, or malignancy type.
The coexistence of malignancy and beta thalassemia is not rare. Any alarming signs and symptoms including worsening anemia, splenomegaly, or lymphadenopathy should be a motive for concern because these symptoms may signal malignant disease.
β地中海贫血是世界上最常见的遗传性疾病之一。本研究的目的是确定伊朗重型β地中海贫血（BTM）和中间型β地中海贫血（BTI）患者恶性肿瘤的发生率、特征和模式。
我们通过对2002年至2007年期间伊朗四个地中海贫血中心的BTM和BTI患者进行回顾性病历审查，开展了一项多中心研究。共评估了4630例地中海贫血患者的记录。使用SPSS软件v.15进行统计分析。P值<0.05被认为具有统计学意义。
我们检测到11例同时患有恶性疾病的β地中海贫血患者。5例（45.4%）被诊断为淋巴瘤，5例被诊断为白血病。BTI患者中癌症患者的比例更高。0至39岁的地中海贫血患者被诊断出患有癌症，但年龄较大的患者中未发现。在地中海贫血患者总体以及BTI患者中，<10岁儿童的癌症发病率年龄特异性最高，而在BTM组中，20至29岁患者的发病率最高。BTM和BTI患者与癌症患者在年龄、性别、脾切除术、血红蛋白、铁蛋白水平或恶性肿瘤类型方面无统计学显著差异。
恶性肿瘤与β地中海贫血并存并不罕见。任何令人担忧的体征和症状，包括贫血加重、脾肿大或淋巴结病，都应引起关注，因为这些症状可能预示着恶性疾病。
