Open AccessEvaluation of Children With Chronic Immune Thrombocytopenic Purpura and Evans Syndrome Treated With Rituximab
Author(s) -
Dilek Gürlek Gökçebay,
Betül Tavil,
Ali Fettah,
Neşe Yaralı,
Fatih Mehmet Azık,
Bahattin Tunç
Publication year - 2012
Publication title -
clinical and applied thrombosis/hemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.643
H-Index - 53
eISSN - 1938-2723
pISSN - 1076-0296
DOI - 10.1177/1076029612451649
Subject(s) - rituximab , medicine , evans syndrome , splenectomy , thrombocytopenic purpura , platelet , immune system , gastroenterology , immunology , pediatrics , antibody , lymphoma , spleen , autoimmune hemolytic anemia
The primary objective was to evaluate the response rate of rituximab therapy for children with chronic immune thrombocytopenic purpura (ITP) and Evans syndrome (ES) and immune reconstitution of these children after rituximab therapy. Eleven patients with chronic ITP and 2 with ES between 6 and 18 years of age and platelet count less than 20 × 10 9 /L received rituximab. Overall response (OR) was defined as an increase in platelet count above 50 × 10 9 /L. The mean age of 13 children (9 girls, 4 boys) was 11.2 ± 3.8 years (6-18). One of the patients with ES had been splenectomized; others were not. The patients mean follow-up time was 10.3 ± 9.3 months after rituximab therapy. Two patients achieved complete response, 4 patients achieved partial response, and OR rate was 46% (6 of 13) after therapy. Seven patients have no response. In conclusion, rituximab may be considered prior to splenectomy in children with chronic ITP and ES with an acceptable toxicity profile.