Exposure to Hydroxyurea During Pregnancy in Sickle‐β Thalassemia: A Report of 2 Cases

From the National Institute of Immunohaematology (Dr Italia, Dr Nadkarni, Dr Sawant, Dr Ghosh, Dr Colah) and the Haematology Department (Dr Jijina, Dr Chandrakala), KEM Hospital, Parel, Mumbai, India. Submitted for publication January 9, 2009; revised version accepted July 2, 2009. Address for correspondence: Roshan B. Colah, MSc, PhD, Scientist F, National Institute of Immunohaematology, 13th Floor, N. M. S. Bldg, KEM Hospital Campus, Parel, Mumbai 400 012, India; e-mail: colahrb@gmail.com. DOI: 10.1177/0091270009343933 T is a high prevalence of the sickle cell gene in malaria endemic areas due to the selective pro­ tection it provides against malaria. Approximately 300 population groups in India have shown the pres­ ence of the β gene, and the prevalence of carriers has varied from 2% to 34%. β thalassemia is also prevalent in India, and the interaction of hemoglo­ bin S and β thalassemia is not uncommon. Although the clinical manifestations of sickle cell anemia and sickle­β thalassemia in India are milder compared to Africans, there are many patients with clinically severe disease. Improved medical facilities and public health care have reduced infant mortality and deaths due to infectious diseases in India, leading to a better life span even in patients with genetic diseases such as sickle cell syndromes. This demands the need for improved management of these patients with severe clinical symptoms through different stages of life. Hydroxyurea (HU) has been a drug of choice in the management of sickle cell disease patients with severe clinical manifestations. It has been shown to reduce the number of vaso­occlusive crises and trans­ fusion requirements in patients on this therapy. However, hydroxyurea is teratogenic in experimental animals but may not necessarily be so in humans. One of the most important and critical times to manage a female patient with severe sickle cell ane­ mia is during pregnancy. Complications arise due to severe maternal anemia, frequent episodes of vaso­ occlusive crisis leading to hypoxia of the placenta, and toxemia of pregnancy. We report 2 female patients with sickle­β thalassemia who were on hydroxyurea therapy and were managed through their pregnan­ cies after discontinuing hydroxyurea therapy.