Azathioprine in Refractory Idiopathic Thrombocytopenic Purpura

Management of Chronic Idiopathic Thrombocytopenic Purpura in Children, with Particular Reference to Immunosuppressive Therapy

“…The finding that purine-antagonists (6-mercaptopurine, thioguanine, and azathioprine) are capable of suppressing immune responses has led many authors to use these agents in the treatment of ITP [3,7,20,31,34,36,47,48]. However, the natural history of untreated ITP has to be taken into account: approximately 80% of the affected children undergo spontaneous remission within 1 to 3 months [37,41,42].…”

Idiopathic Thrombocytopenia after Treatment with Steroids and Immunosuppressives

“…Because no apparent clinical remission followed splenectomy, and long-term application of moderate steroid dosages of 0.5 mg/kg/day did not produce a favorable response -on the contrary the purpura of our pa tients returned -the steroids were conlinously replaced by azathioprine. This drug for immune suppression was chosen for its lowest rate of side effects [15]. Since our patients did not complain of vomiting, dizziness, lack of appetite or weight loss, and serious complications such as anemia, pancytopenia, aplastic syndrome, icterus or cholestasis were not ob served after 6 months of azathioprine therapy, we discontinued the ster oids and maintained the patients on azathioprine alone, with recom mended doses of up to 100 mg daily.…”

“…The term autoimmune thrombocytopenic purpura should be applied to patients fulfilling these above-mentioned criteria after exclusion of other clinically similar disorders, such as systemic lupus erythematodes [5,8,14,18]. The effect of treatment of this autoimmune disorder appeared unsatisfactory in about 50% of adults and 10% of children, who had re ceived therapeutic attempts with splenectomy and steroids [15]. Of these two therapeutic possibilities, better results were obtained from a splenectomy with immediate response when scanning revealed the spleen as the predominant location of platelet destruction [17].…”

“…Since chronic ITP has been included among the autoimmune diseases despite the lack of detectable thrombocytic isoantibodies in about 60% of the cases, it has been suggested that refractory ITP should be treated with drugs capable of suppressing the immune response [2,13,15]. This re port is concerned with the observation of three patients with refractory ITP, who have been splenectomized in vain.…”

How I treat refractory immune thrombocytopenia

“…72 Smaller series also showed favorable results. 73,74 One study of 6-mercaptopurine in pediatric autoimmune cytopenias has been published. In that series of 29 patients, there was an 83% response rate, yet only 4 children (2 with ITP) remained in remission without additional immunosuppressive therapy.…”