Hydroxyurea (HU) is an oral drug that ameliorates the clinical course of,si
ckle cell anemia by increasing the levels of fetal hemoglobin and decreasin
g the adhesion of red cells to endothelium. Although HU has minimal short-t
erm toxicity, few data are available about the long-term safety and the pot
ential risk for carcinogenesis or leukemogenesis. An 8-year-old child with
sickle cell/beta (0)-thalassemia who received HU treatment for painful cris
es is described. Six months after the initiation of the HU treatment he dev
eloped Hodgkin's disease, lymphocyte predominance subtype. Chemotherapy, in
duced a complete remission. After discontinuation of chemotherapy the painf
ul crises recurred and bone marrow transplantation was decided at the age o
f 12 years. Two years after the bone marrow transplantation, the child is i
n complete remission without painful crises. Although the authors suggest t
hat the development of Hodgkin's disease is a coexisting event, questions a
rise about the safely of HU treatment in childhood.