Hematological malignancies complicating β-thalassemia syndromes: a single center experience

which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Hematological malignancies complicating β-thalassemia syndromes: a single center experience TO THE EDITOR: Only few reports have addressed the occurrence of hematological malignancies in patients suffering from β-thalassemia. We herein report two patients with major and intermedia β-thalassemia who were complicated with Hodgkin's lymphoma and chronic myelogenous leuke-mia (CML) during the course of the disease, respectively. This report indicate that malignancies should be suspected and kept in mind in patients with β-thalassemia syndromes presenting with proposed signs and symptoms including unexplained lymphadenopathy, leukocytosis and splenom-egaly. β-thalassemia major is a genetic hemoglobinopathy characterized by point mutations of β-globin chain resulting in accumulation and deposition of unpaired β-globin chains in red blood cell (RBC). This causes severe hemolysis and ineffective hematopoiesis leading to severe anemia [1]. Currently the availability of proper care and appropriate treatment strategies including regular transfusions, alloge-neic stem-cell transplantation, inducers of fetal hemoglobin, and precise iron chelation therapy with desferrioxamine (DFO) and/or deferiprone (DFP) have resulted in increased life expectancy and quality of life of the patients suffering from β-thalassemia major [2-4]. This increased life span increases the possibility of occurrence of other diseases such as malignancies. Although, previously malignancies were considered rare in β-thalassemia patients, but currently the number of malignancies reported in thalassemic patients is increasing [5-9]. We herein, report two patients with β-thalassemia major and intermedia who developed Hodg-kin's lymphoma and chronic myelogenous leukemia (CML) during the course of the disease. A 16-year-old girl with β-thalassemia major presented with painless bilateral cervical lymphadenopathies for 1 month. She had been on regular transfusion since 9 months of age and was HCV positive due to regular transfusions. Her parents were heterozygotes for β-thalassemia minor. Initial evaluations including complete blood count, biochemistry tests, Epstein barr virus infection (EBV) and tox-oplasmosis studies were not conclusive. white blood cells and platelets were normal. serum ferritin levels of the patient were 3,200 ng/mL and 3,000 ng/mL at two occasions. She was receiving subcutaneous desferrioxamine at least 5 nights/week as iron chelation since 2.5 years of age. After about 3 weeks of persistent cervical lymphadenopathy, computerized tomography (CT) scan of the neck and chest was performed revealing bilateral cervical and upper anterior mediastinal lymph nodes with para-tracheal involvement .Abdominopelvic imaging was normal. An excisional lymph node biopsy was performed which was compatible with Hodgkin's lymphoma, nodular …