Hydroxyurea and blood transfusion therapy for Sickle cell disease in South Asia: inconsistent treatment of a neglected disease

Abstract

Background Hydroxyurea and blood transfusion therapies remain the main therapeutic strategies for Sickle cell disease. Preliminary data suggest substantial variation and inconsistencies in practice of these two therapeutic modalities in South Asia. In this systematic review we searched Medline, Cochrane library and Scopus for articles on usage of hydroxyurea and blood transfusion therapies for sickle cell disease in South Asia published in English between October 2005 and October 2020. Results We selected 41 papers: 33 from India, 3 from Sri Lanka, 2 each from Pakistan and Bangladesh and one from Nepal. Only 14 prospective trials focused on hydroxyurea therapy from which majority (n = 10; 71.4%) adopted fixed low dose (10 mg/kg/day) regimen. With hydroxyurea therapy, 12 and 9 studies reported significant reductions in vaso-occlusive crises and transfusion requirement respectively. Severe anaemia (haemoglobin level < 6–7 g/dl) was the commonest indicator (n = 8) for transfusion therapy followed by vaso-occlusive crisis. Conclusions Published data on the hydroxyurea and transfusion therapies in South Asia are limited and heterogeneous. A clear gap of knowledge exists about the nature of the sickle cell disease in the Indian subcontinent particularly from countries outside India necessitating further evidence-based assessments and interventions.

Sickle cell anemia: a mimicker of rheumatoid arthritis

“…Fixed-low dose hydroxyurea treatment (10 mg/kg/day) has shown encouraging outcomes in Indian SCD patients and may be appropriate for sickle patients from other South Asian nations who are of Indian descent. 9 Ataga et al conducted a randomized controlled trial of 198 sickle cell anemia patients and compared the efficacy of crizanlizumab with placebo and found that crizanlizumab therapy resulted in a significantly lower rate of sickle cell-related pain crises than placebo and was associated with a low incidence of adverse events. 10 It could be a potential treatment option for our patient for the prevention of further episodes of vaso-occlusive crisis.…”

Sickle Cell Disease Update: New Treatments and Challenging Nutritional Interventions

“…This small-molecule drug is able to attach to and stabilize hemoglobin, preventing hemoglobin polymerization (i.e., formation of abnormal hemoglobin) that causes the formation of sickle shaped red blood cells [ 88 ]. In well-resourced countries, three potential treatments are available for preventing or reducing the morbidity and mortality associated with SCA: transfusions, hydroxyurea, and stem cell transplantation [ 89 ]. There is no evidence of any benefits of corticosteroid use in SCD acute events [ 90 ].…”

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“…Malaria parasites, however, are now resistant to these drugs [ 9 ]. Hydroxyurea therapy for sickle cell disease has been utilized for many years and appears to be helpful for both the main and secondary prevention of stroke in sickle patients [ 12 ]. It does so by elevating the HbF levels that are present in the RBCs of patients.…”