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Hemato-Oncology

                                               P-HO-028
                             FVIII Inhibitors in Patients with Hemophilia A
                                 at Haji Adam Malik Hospital Medan

                                     Olga Rasiyanti Siregar, Bidasari Lubis
              Department of Child Health, Faculty of Medicine Universitas Sumatera Utara, Medan, North Sumatera, Indonesia

                                               Abstract
            Background Estimated 1 in 5000 male has hemophilia A. FactorVIII (FVIII) replacement therapy, which
            is given monthly as prophylaxis in pediatric patients with hemophilia A in Haji Adam Malik Hospital
            Medan, can induce the development of antibodies (inhibitors)  to exogenous FVIII which will complicate
            management by limiting its effectiveness in stopping and/or preventing bleeding episodes, resulting in
            excessive bleeding or unusual bleeding sites and inadequate recovery. Objective To analyze the association
            between the incidence of FVIII inhibitors and several risk factors in hemophilia A patients. Methods This
            is cross sectional study. Pediatric patients with hemophilia A who received prophylactic FVIII replacement
            routinely at Haji Adam Malik Hospital Medan were included. Patients with severe bleeding episodes or
            other emergency conditions were excluded. 28 patients came under the criteria and were tested for FVIII
            inhibitors with Bethesda assay. Data were analyzed using SPSS version 23.0. Results There were 10 mild,
            12 moderate, 6 severe hemophilia A patients with median age 9 (range 1-7) years and median hemophilia A
            duration 5 (range 1-12) years. 23 patients had no inhibitor, 1 with low titre, 4 with high titre FVIII inhibitors.
            Only duration of being diagnosed hemophilia A was associated with incidence of FVIII inhibitors (P=0.013).
            All patients with inhibitors had been diagnosed as hemophilia A for more than 5 years. Age, hospitalization
            history, history of recombinant therapy, surgical history, hemophilia A severity, and bleeding history were
            not associated with FVIII inhibitors. Conclusion Duration of being diagnosed as hemophilia A is associated
            with incidence of FVIII inhibitors.
                            Keywords: hemophilia A; factor VIII inhibitors; incidence, risk factors


                                               P-HO-029
                         Burkitt’s Lymphoma in 3-year-old boy: A Case Report

                 Rizaldo Bagoes Dinatha, Andi Cahyadi, Maria Chistina Shanty Larasati, Mia Ratwita Andarsini, I
                                Dewa Gede Ugrasena, Bambang Permono, Dyah Fauziah
              Department of Child Health, Faculty of Medicine Universitas Airlangga/Dr. Soetomo General Academic Hospital,
                                         Surabaya, East Java, Indonesia

                                               Abstract
            Background Burkitt’s lymphoma is a highly aggressive non-Hodgkin B-cell lymphoma, and the fastest growth
            human tumor, classified as endemic type, sporadic type, and immunodeficiency-associated type. Burkitt’s
            lymphoma is usually diagnosed in young adults with predominantly in male presented with facial swelling
            and intraoral exophytic mass. Burkitt’s lymphoma is rare in young children. Early diagnosis and prompt
            treatment will increase the survival rate. Objective To report a case of Burkitt’s lymphoma in young children.
            Case A 3-year-old boy came with left maxillary mass for 3 months ago, fixed, painless, without lymph nodes
            enlargement accompanied by sub-febrile, weight lost without bone pain. The mass was progressively enlarged,
            very painful in the last 2 weeks, infiltrated left eye and caused difficulty in opening the mouth. Complete
            blood count within normal limit without anemia, thrombocytopenia, leukocytosis, nor lymphocytosis. The
            serum LDH increase to 1260 U/L. Histopathological examination support non-Hodgkin lymphoma with
            the starry sky appearance. Leukemic cell or cancer infiltration were not found in bone marrow aspiration.
            He was treated with chemotherapy combination of COP/COMP/CYM using cyclophosphamide, vicristine,
            methotrexate, cytarabine and oral prednisone. The mass resolved after 3 weeks evaluation with minimal
            complication. Conclusion Burkitt’s lymphoma diagnosis based on clinical, laboratory, and histopathological
            findings. Early diagnosis and chemotherapy had better outcome.
                               Keywords: maxillary mass; Burkitt’s  lymphoma;  young child







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