Abstract

The management of chronic refractory ITP continues to be a frustrating experience for the patients as well as treating hematologists. Many patients relapse after steroid therapy as well as splenectomy. Even though latter modality leaves them susceptible to life-threatening infections, without making them symptom free. Immunoglobulins and anti-D vaccine do show a good but brief response. Retuximab is a newer drug to be used in ITP, but it is out of reach for most of the patients in a developing country like India. Of late Dapsone has been seen to be an effective drug in such situations and probably acts through reticulo-endothelial blockade.

We report the efficacy of Dapsone in 46 consecutively treated patients in our hospital, who had continued to be symptomatic despite adequate steroid therapy. They were chosen to be treated with Dapsone without being subjected to splenectomy. Their median age was 21 years(range : 8–46 years). The patients having associated connective tissue disorders were excluded. All the patients were screened for hemolytic tendency and splenectomy. Dapsone was started at an initial dose of 50 mg/day and escalated to100 mg/day, if the response was sub-optimal. Children received 25 mg/day initially and subsequent dose was adjusted to a minimal level required to achieve symptom free status. The aim of the therapy was to eliminate purpura rather than to achieve a particular level of platelet count. The duration of treatment ranged from six months to 48 months.36 patients (80%) showed an overall response with disappearance of purpura and a significant rise (> 40,000/cmm) in platelet count. Treatment was well tolerated and no adverse effects were encountered even though dose related hemolysis has been reported. The drug was continued in 36 responders for a median period of 32 months and they all continued to be asymptomatic throughout. The treatment was intentionally withdrawn in 12 responders, but thrombocytopenia recurred in all these patients. In our experience Dapsone provided an inexpensive and well tolerated alternative for patients with ITP who showed suboptimal response to steroid therapy. The splenectomy was successfully avoided in these patients. Its use is cost effective as well. Both these factors make this a great boon to the patients in a developing country. We recommend that all patients be started on Dapsone once they fail to show response to steroids. Splenectomy should be reserved for the patients who do not respond to Dapsone.

Disclosure: No relevant conflicts of interest to declare.

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