Dapsone for chronic idiopathic thrombocytopenic purpura in children and adults – a report on 90 patients

Damodar, Sharat; Viswabandya, Auro; George, Biju; Mathews, Vikram; Chandy, Mammen; Srivastava, Alok

doi:10.1111/j.1600-0609.2005.00545.x

Cited by 58 publications

(72 citation statements)

References 19 publications

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“…Intensive consolidation chemotherapy for acute myeloid leukemia (AML) patients in complete remission is being increasingly administered on an outpatient basis. Although this approach has been found to be safe and feasible in younger patients [1][2][3][4][5][6], its safety in older patients remains unknown. We therefore undertook an evaluation of outpatient-based consolidation chemotherapy in older AML patients, and compared results to younger patients treated at the same institution over the same time period.…”

Section: Methodsmentioning

confidence: 99%

“…This agent is contraindicated in patients with G6PD deficiency and has a good safety profile. Hemolytic anemia and an increase of methemoglobin (MHb) are the main reported side effects [8,9].Previous studies highlighted the therapeutic activity of dapsone as salvage therapy in primary ITP with 40-60% overall response rate and 15-50% CR rate [1][2][3][4][5][6]. The response to dapsone was unaffected by pretreatment characteristics such as sex, age, platelet count, or duration of ITP and was generally treatment dependent, even if the persistence of response after dapsone discontinuation has been registered in some patients.…”

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confidence: 99%

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Dapsone salvage therapy for adult patients with immune thrombocytopenia relapsed or refractory to steroid and rituximab

et al. 2011

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Dapsone is an antibacterial sulfonamide with anti-inflammatory property, which showed therapeutic activity in patients with immune thrombocytopenia (ITP) [1][2][3][4][5][6]; the activity in patients who showed refractoriness to rituximab is unknown. We evaluated the effect of dapsone in 20 consecutive adult patients, median age 51 years, with primary ITP previously treated at least with steroids and rituximab. Median baseline platelet count was 19 3 10 9 /L, and the median interval between diagnosis of ITP and dapsone therapy was 46 months. Response (platelet count 30 3 10 9 /L) and complete response (CR; platelet count 100 3 10 9 /L) were 55 and 20%, respectively; median time to response (TTR) was 1 month. All responders were able to interrupt any other specific anti-ITP treatment. The median duration of dapsone therapy in responders and the median response duration were 31 and 42 months, respectively. None of responders lost response during treatment. One patient in CR interrupted dapsone after 9 months and still maintained the response after 48 months. None of the patients interrupted the treatment for toxicity. All the patients were screened for normal glucose-6-phosphate-dehydrogenase (G6PD); two patients showed mild increase of methemoglobin (MHb). These results highlight the therapeutic activity and good safety profile of dapsone in patients with ITP who previously failed rituximab treatment. ITP is an acquired autoimmune disease characterized by increased platelet destruction, impaired megakaryocyte maturation with reduced platelet production, and possible hemorrhagic complications. Treatment is generally indicated for symptomatic patients, that is, those with active bleeding or very low platelet count (usually < 20-30 3 10 9 /L). Glucocorticoids, potentially associated with intravenous high-dose immunoglobulin in those cases with high risk of major bleeding, represent the standard first line of treatment [7]. However, despite the high initial therapeutic efficacy, in most cases, steroids tapering or withdrawal is followed by a drop in platelet count and the need for additional treatment. Treatment of patients with relapsed or refractory symptomatic ITP, particularly of those who are not eligible for, refused or failed splenectomy, still lacks a gold standard therapy. Dapsone is an antibacterial sulfonamide synthesized in 1908 with antimicrobial effect against leprosy, pneumocystic Jiroveci pneumonia in AIDS, toxoplamosis, and malaria. Since 1950s, dapsone also was recognized to have activity in a number of noninfectious inflammatory diseases of the skin as dermatitis herpetiformis and blistering disorders. The anti-inflammatory effect of dapsone is not fully understood, but it appears at least targeted against neutrophils, with interference of myeloperoxidase, inhibition of lysosomal enzymes, chemotaxis, and integrin-mediated adherence function. This agent is contraindicated in patients with G6PD deficiency and has a good safety profile. Hemolytic anemia and an increase of methemoglobin (MHb) are the ma...

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Dapsone salvage therapy for adult patients with immune thrombocytopenia relapsed or refractory to steroid and rituximab

et al. 2011

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“…A retrospective analysis of dapsone in chronic or persistent ITP that included 35 children demonstrated a response rate of 66% and continuous complete response rate (maintenance of a platelet count Ͼ 50 ϫ 10 9 /L with or without dapsone) of 31%. 48 Studies of thrombopoietin receptor agonists in children and adolescents are under way, but results have not been published. Thus, no recommendations for the use of these agents can be made at this time.…”

Section: 3b We Suggestmentioning

confidence: 99%

The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia

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Lim

Crowther

et al. 2011

Blood

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Immune thrombocytopenia (ITP) is commonly encountered in clinical practice. In 1996 the American Society of Hematology published a landmark guidance paper designed to assist clinicians in the management of this disorder. Since 1996 there have been numerous advances in the management of both adult and pediatric ITP. These changes mandated an update in the guidelines. This guideline uses a rigorous, evidence-based approach to the location, interpretation, and presentation of the available evidence. We have endeavored to identify, abstract, and present all available methodologically rigorous data informing the treatment of ITP. We provide evidence-based treatment recommendations using the GRADE system in those areas in which such evidence exists. We do not provide evidence in those areas in which evidence is lacking, or is of lower quality-interested readers are referred to a number of recent, consensus-based recommendations for expert opinion in these clinical areas. Our review identified the need for additional studies in many key areas of the therapy of ITP such as comparative studies of "front-line" therapy for ITP, the management of serious bleeding in patients with ITP, and studies that will provide guidance about which therapy should be used as salvage therapy for patients after failure of a first-line intervention. (Blood. 2011;117(16):4190-4207)

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“…Dapsone was found to be an effective, inexpensive, and well-tolerated drug for chronic ITP when steroids or other immunosuppressants failed. [7] Danazol, a category X drug, is best avoided in pregnancy due to possible pseudohermaphroditism and virilization. [1] In a retrospective review, 129 pregnancies exposed to danazol resulted in 94 live children.…”

Section: 2] Dapsone Danazol and Intrapartum Splenectomy In Refracmentioning

confidence: 99%

Dapsone, danazol, and intrapartum splenectomy in refractory ITP complicating pregnancy

Varghese¹,

Viswabandya²,

Mathew³

2008

Indian J Med Sci

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Dapsone for chronic idiopathic thrombocytopenic purpura in children and adults – a report on 90 patients

Cited by 58 publications

References 19 publications

Dapsone salvage therapy for adult patients with immune thrombocytopenia relapsed or refractory to steroid and rituximab

Dapsone salvage therapy for adult patients with immune thrombocytopenia relapsed or refractory to steroid and rituximab

The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia

Dapsone, danazol, and intrapartum splenectomy in refractory ITP complicating pregnancy

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