β-Thalassemia and Lymphoma: coincidence or association? – a clinical report

Abstract

β-Thalassemia is one of the most common hemoglobinopathies
in the world, characterized by the absence or decrease on the
β-hemoglobin chain production.
The coexistence of β-Thalassemia and lymphoma is rare, but it
has been reported in the literature, associated with better treatment approaches of β-Thalassemia major.
In this article, it is reported a case of β-Thalassemia minor
presented with non-Hodgkin lymphoma, representing the first
documented case in Portugal and the second worldwide.
Several factors, still controversial, may contribute to the pathogenesis of malignant disease in β-Thalassemia: infections, iron
overload, immunomodulation and more intensive hematopoiesis
to compensate ineffective erythropoiesis and chronic hemolysis,
increasing the probability of mutations, including oncogene expression. Based on a literature review, it is argued that the term
that most likely relates these two entities is “association” and
not “coincidence”.