Risk of bleeding and inhibitor development after circumcision of previously untreated or minimally treated severe hemophilia A children.

BACKGROUND: Surgery and intensive factor VIII (FVIII) replacement may be risk factors for development of inhibitors. OBJECTIVE: To evaluate time and rate of inhibitor development postcircumcision over 12-month period, and to assess bleeding of children with severe hemophilia A after low-dose FVIII replacement and local hemostasis. PATIENTS AND METHODS: Sixty-one previously untreated patients (PUPs) or minimally treated patients (MTPs) with severe hemophilia A less than 36 months were enrolled; 25 underwent circumcision during the 18-month enrollment period, and 36 matched patients were not circumcised. All patients were treated on demand with plasma-derived FVIII, and all were inhibitor negative at the time of enrollment. Intron 22 inversion was analyzed. A potent hemostatic agent (gelatin sponge) was applied on the site of surgery, and then dressed with gauze. Two doses of FVIII concentrate (25 U/kg) were given, 1 hour before circumcision and 1 hour before removal of dressing. The inhibitor was determined every 8 exposure days (EDs). RESULTS: None of the patients had bleeding or infection, except one who had minimal transient bleeding 8 days after surgery, and was treated easily by a single dose of FVIII (50 U/kg). After a median of 16 EDs, high-titer inhibitors developed in seven patients: three patients in the circumcised group (12%) in contrast to four patients (11.1%) in the noncircumcised group. CONCLUSION: Two doses factor concentrate and gelatin sponge application were generally enough to prevent bleeding after circumcision of severe hemophilia A. Circumcision and low-dose FVIII protocol were not an additional risk for development of high-titer inhibitor.

Red blood cell alloimmunization in transfusion-dependent Egyptian patients with thalassemia in a limited donor exposure program.

BACKGROUND: Thalassemia is considered the most common hemoglobinopathy in Egypt and is one of its major health problems. Lifelong red blood cell (RBC) transfusion remains the main treatment for severe forms; however, RBC alloimmunization results as a complication of regular transfusions due to repeated exposure to foreign antigens. The objective was to compare the frequency of alloantibodies in a group of patients in a limited donor exposure program (LDEP) with those receiving RBCs from multiple donors in Egyptian transfusion-dependent patients with thalassemia. STUDY DESIGN AND METHODS: A total of 235 regularly transfused patients with thalassemia were studied, 36 of which were on LDEP. All patients were investigated for the presence of RBC autoantibodies and alloantibodies, followed by antibody identification for positive patients. RESULTS: Forty-six (19.5%) patients developed RBC alloantibodies. The most common clinically significant alloantibodies were directed against antigens in the Kell and Rh systems. Development of alloantibodies was associated with older age, higher transfusion frequency, and splenectomy. A trend toward lower alloimmunization was elicited in the LDEP group, where 8.3% (3/36) patients were alloimmunized compared to 21.6% (43/199) in the non-LDEP one (p=0.057). CONCLUSIONS: Examination of donor RBCs for presence of Kell and Rh(E) antigens before transfusion can help decrease RBC alloimmunization. Further larger studies are required to assess the frequency of alloantibody production in patients on LDEP.