Hematological malignancies complicating β-thalassemia syndromes: a single center experience
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 toxoplasmosis 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 sclerosis subtype. Clinical staging revealed stage 2A (spleen was considered enlarged due to thalassemia since it was not a new finding). She was scheduled to receive systemic chemotherapy with 6 alternating cycles of MOPP/ABVD followed by radiotherapy and was off-therapy thereafter. Her mother also developed colon adenocarcinoma 6 years later and her brother (who was hematologically normal) died of brain tumor 8 years later. She also received antiviral treatments including α-interferon and ribavirin for HCV and changed to be negative. Fortunately, the patient is in remission for Hodgkin's lymphoma and still on regular blood transfusion after 10 years of follow-up.