Malignancies in patients with beta-thalassemia major and beta-thalassemia intermedia: a multicenter study in Iran

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.