Sickle Cell Diseases and Hematological Maligancies

Hematological malignancies including lymphoblastic lymphoma, multiple myeloma and hairy cell leukemia have been reported in patients with sickle cell

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Sickle Cell Diseases and Hematological Maligancies
Sickle cell diseases (SCD), due mostly to homozygous hemoglobin S (HbS) or compound heterozygosity of HbS and HbC or -thalassemia, are multisystem disorders manifested by a variable degree of anemia, acute vaso-occlusive episodes, and chronic organ damage. The mutations involved are not directly oncogenic. However, SCD is associated with high proliferative rates of bone marrow cells, chronic inflammation, and immune dysregulation all of which could predispose to malignancy. Fear of increased malignancy is one of the barriers to more widespread use of hydroxurea, the only drug therapy demonstrated to alter the course of SCD. Small case series and surveys have hinted at increased cancer incidence amongst SCD patients, but no examination of population-based data has been reported. Malignancy has previously been reported in cases of congenital hemolytic anemia (SCD, thalassemia, hereditary spherocytosis and Hemoglobin C disease)1. Hematological malignancies including lymphoblastic lymphoma, multiple myeloma and hairy cell leukemia have been reported in patients with sickle cell disease2.

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