Abstract Ref Number = APCP923
CORRELATION BETWEEN SEVERITY OF HAEMOPHILIA A IN CHILDREN AND IRON DEFICIENCY ANEMIA
Aini Ariefa Bahzar,Andi Cahyadi,Maria Christina Shanty Larasati,Mia Ratwita Andarsini,I Dewa Gede Ugrasena,Bambang Permono
Department of Child Health Faculty of Medicine Universitas Airlangga Dr Soetomo Hospital Surabaya Indonesia
Background : Haemophilia A was an X-linked recessive disorder characterized by factor VIII deficiency resulting in lifelong bleeding. Recurrent bleeding especially hemarthrosis lead to iron deposition in membrane synovial that become iron deficiency. Increased synovial iron stores in children with haemophilia cannot possibly compensate for the iron deficiency resulting from external blood loss and the depletion of normal storage compartments such as the liver and the bone marrow. The aim of this study is to correlate between severity in haemophilia A children and iron deficiency anemia.
Material : A cross-sectional study evaluated children with haemophilia A below 18 years in Pediatric Hematology Oncology, Dr. Soetomo Hospital from January to March 2018. Severity of haemophilia A was classified based on factor VIII level, with 5-30% as mild, 1-5% as moderate and <1% as severe. We evaluated iron deficiency anemia among children with haemophilia A. Iron deficiency anemia was defined according to WHO (low haemoglobin levels based on age, serum iron <50µg/dl, and transferin saturation (TSAT) <15%). The Goodman and Kruskal's gamma correlation test was use to data analysis (P=0.05).
Results : A total 25 patient enrolled with median age 12 (2-18) years, classified as mild (15), moderate (8), and severe (2) haemophilia. Iron deficiency anemia was found in 7/25 children with classified as mild (1/7), moderate (4/7), and severe (2/7) haemophilia. The median haemoglobin levels was 10.3 (9-10.5) g/dL, median corpuscular hemoglobin 22.9 (19.5-29.8) pg. The median serum iron level was 18 (15-32) µg/dL, total iron binding capacity (TIBC) was 143 (130-242) µg/dL, and TSAT was 12.3 (8.1-14.8)%. There was significant correlation between haemophilia severity and iron deficiency anemia (r=0.917, P = 0.001).
Conclusions : Severity hemophilia A has strong correlation with iron deficiency anemia
Keywords: haemophilia A children severity iron deficiency anemia