Dapsone is NOT used to treat sickle cell disease
Dapsone has no established role in the management of sickle cell disease and should not be used for this indication. The evidence provided discusses dapsone exclusively in the context of other conditions—immune thrombocytopenia (ITP), hidradenitis suppurativa, bullous pemphigoid, and pemphigus vulgaris—but never mentions sickle cell disease as an indication 1.
Established Treatments for Sickle Cell Disease
The evidence clearly identifies the following as appropriate therapies for sickle cell disease:
First-Line Therapy
- Hydroxyurea remains the primary disease-modifying therapy for sickle cell disease, recommended for all patients with HbSS or HbSβ0-thalassemia genotypes starting as early as 9 months of age 2, 3, 4.
- Hydroxyurea increases fetal hemoglobin and reduces red blood cell sickling 3.
Additional FDA-Approved Therapies
- L-glutamine (Endari) is approved for patients 5 years and older to reduce pain events by reducing oxidative stress in red blood cells 2, 3.
- Crizanlizumab (anti-P-selectin monoclonal antibody) reduced pain crises from 2.98 to 1.63 per year compared with placebo 3, 5.
- Voxelotor increased hemoglobin by at least 1 g/dL in 51% vs 7% with placebo 3, 5.
Curative Options
- Hematopoietic stem cell transplantation is the only curative therapy, with best results in children with matched sibling donors 3, 4, 6.
Why Dapsone is Not Appropriate
Dapsone's mechanism of action—inhibiting neutrophil myeloperoxidase and causing hemolysis by overwhelming G6PD reductive capacity—is designed to saturate macrophage phagocytic potential in conditions like ITP 1. This mechanism would be contraindicated in sickle cell disease, where patients already experience chronic hemolysis and anemia. Inducing additional hemolysis would worsen the underlying pathophysiology of sickle cell disease 3.
Critical Safety Concern
- Patients with G6PD deficiency are at particular risk for significant hemolysis with dapsone 1.
- G6PD deficiency has higher prevalence in populations also affected by sickle cell disease (males of African ancestry) 1.
- Adding dapsone-induced hemolysis to the baseline hemolysis of sickle cell disease would be harmful.