Dapsone: An Old but Effective Therapy in Pediatric Refractory Immune Thrombocytopenia
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ORIGINAL ARTICLE
Dapsone: An Old but Effective Therapy in Pediatric Refractory Immune Thrombocytopenia Sanjeev Khera1 • Suman Kumar Pramanik2 • Uday Yanamandra2 Kundan Mishra2 • Rajan Kapoor3 • Satyaranjan Das4
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Received: 25 November 2019 / Accepted: 27 April 2020 Ó Indian Society of Hematology and Blood Transfusion 2020
Abstract There are no definitive guidelines for management of chronic or refractory immune thrombocytopenia (ITP) in children. Dapsone is an inexpensive and efficacious, yet neglected, therapeutic option for treatment of chronic ITP. We evaluated the efficacy and safety of dapsone in the management of chronic ITP in children. Children with chronic ITP \ 14 years with minimum grade 2 bleeds refractory to either splenectomy/rituximab/ eltrombopag; who were offered dapsone therapy were retrospectively analyzed. Dapsone intolerance and G6PD deficiency were excluded. Dapsone was started at a dose of 1–2 mg/kg/day. Response to dapsone as per international working group definitions, time to response along with side-effects were noted. Forty-four children enrolled; 29 analyzed. Nineteen were refractory to rituximab, 8 to splenectomy and 6 to eltrombopag. Median age was 9.8 years (3–14) with 16/29 males. Median dapsone dose was 1.59 mg/kg/day (range 1–2.1). Overall response was seen in 21/29 (72%): Complete Response in 7/29 (24%), Partial Response in 14/29 (48%). All responses were sustained for minimum 3 months. Median duration to response was 2.9 months (2–6.6). Median follow up was 28 months (6–73) and relapse rate-21%. Major side effects & Sanjeev Khera [email protected] 1
Department of Pediatrics, Army Hospital Research and Referral, Delhi 110010, India
2
Department of Hematology, Army Hospital Research and Referral, Delhi, India
3
Department of Hematology, Command Hospital EC, Kolkata, India
4
Department of Hematology, Command Hospital SC, Pune, India
noted: Methemoglobinemia-01, skin ulceration-02. In three cases dapsone could be tapered and stopped without relapse. Dapsone is an economical and efficacious agent with good safety profile in childhood chronic/refractory ITP. Keywords Dapsone Immune thrombocytopenia Chronic ITP Refractory ITP Abbreviations ITP Immune thrombocytopenic purpura TPO-R Thrombopoeitin receptor G6PD Glucose 6 phosphate dehydrogenase CR Complete response PR Partial response NR No response IWG International working group CTCAE Common terminology criteria for adverse events HRQoL Health related quality of life
Introduction Chronic immune thrombocytopenia (ITP) comprises about 20% of primary ITP in children [1]. In absence of robust guidelines, management of chronic ITP poses challenges to pediatric hematologists. Dapsone is being used in general clinical practice in the management of ITP. More than 80% children with ITP have spontaneous resolution making it difficult to draw any conclusion regarding the efficacy of dapsone in acute or persistent ITP. However, dapsone is an inexpensive and efficacious yet neglected therapeutic
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Indian J Hematol Bl
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