The sulfone syndrome secondary to dapsone prophylaxis in a patient undergoing unrelated hematopoietic stem cell transplantation

Abstract

Dapsone is commonly used for pneumocystis carinii pneumonia (PCP) prophylaxis in immunocompromised patients. It has been used as an alternative therapy in the hematopoietic stem cell transplant (HSCT) setting in patients who can't tolerate trimethoprim-sulfamethoxazole. The Sulfone syndrome is not a well-known sequela of dapsone therapy and occurs at various doses, ranging from 50-300 mg/d. In all cases the syndrome occurs within 2 months of initiating therapy. Its clinical manifestations include: fever, methemoglobinemia, hemolytic anemia, exfoliative dermatitis and transaminits. A 51-year old female underwent a matched unrelated hematopoiectic stem cell transplant for acute mylogenous leukemia. Dapsone therapy was initiated on day +28 at a dose of 100 mg/day for PCP prophylaxis secondary to the patient's history of a sulfonamide allergy. On day +59, one month after initiation of therapy she developed hepatitis, hemolytic anemia, fever and methemoglobinemia of 8%. She was transferred to the intensive care unit and subsequently developed an exfoliative dermatitis. We conclude that the clinical presentation of this patient after HSCT on dapsone therapy coincide with the sulfone syndrome not previously described in a patients after HSCT.

Dapsone induced methemoglobinemia in a patient with glioblastoma

“…Dapsone is metabolized and bioactivated in the liver to a potent, oxidizing hydroxylamine [11]. This dapsone hydroxylamine can then rapidly deplete erythrocyte gluatathione levels, and directly increase the rate of oxidation of hemoglobin to methemoglobin [8, 9, 11, 12]. Methemoglobin is unable to bind oxygen and shifts the oxygen saturation curve to the left, resulting in a decrease in oxygen delivery to tissues which results in cyanosis, dyspnea and tachycardia.…”

Pneumocystis carinii Pneumonia Prophylaxis

“…When dapsone was stopped, the symptoms resolved (Williams et al, 2005). A case report of a patient who died from sulfone syndrome after receiving dapsone prophylaxis post-SCT reported the patient had fever, methemoglobinemia, hemolytic anemia, exfoliative dermatitis, and transaminitis that lead to multisystem organ failure (Abidi, Kozlowski, Ibrahim, & Peres, 2006). These side effects can occur at various doses, most often occur within 2 months of starting the drug, and can even appear as late as 2 weeks after discontinuing the drug (Abidi et al, 2006).…”

“…A case report of a patient who died from sulfone syndrome after receiving dapsone prophylaxis post-SCT reported the patient had fever, methemoglobinemia, hemolytic anemia, exfoliative dermatitis, and transaminitis that lead to multisystem organ failure (Abidi, Kozlowski, Ibrahim, & Peres, 2006). These side effects can occur at various doses, most often occur within 2 months of starting the drug, and can even appear as late as 2 weeks after discontinuing the drug (Abidi et al, 2006). With an increased risk of sulfone syndrome and potential oxidative drug interactions (Williams et al, 2005), dapsone should not be used in patients with glucose-6-phosphate dehydrogenase deficiency (“Cotrimoxazole Is Generally Used to Manage,” 2008).…”