G109(P) Review of paediatric haemophilia in Kuwait

Abstract

IntroductionHaemophilia (A, B) is an uncommon inherited disorder worldwide. This study is a review of paediatric haemophilia in Kuwait.MethodThis was a cross-sectional study involving all the haemophilia (A, B) patients currently followed in the paediatric haematology clinics in Mubarak, Amiri and Adan hospitals in Kuwait. Informed consent was obtained as appropriate. Patients’ initial presentation, clinical findings, family history, laboratory data, clinical course, management and complications were documented.ResultThere were 30 patients – 20 haemophilia A and 10 haemophilia B. Of the former, 10 (50%) presented before 6 months of age, 7 (35%) between 6 months to 2 years, 3 (15%) between 2 – 6 years. Five (50%) of haemophilia B presented between 6 months and 2 years, two (20%) between the age 2 – 6 years, two patients after 6 years and one at birth.Nine (45%) of the haemophilia A patients presented with prolonged bleeding, 3 (15%) with mucous membrane bleeding and 3 with hemarthrosis. Two had spontaneous CNS bleeding, and one with intramuscular bleeding.Five (50%) haemophilia B patients presented with mucous membrane bleeding; 30% were diagnosed during routine screening. The rest one with hemarthrosis and one patient with prolonged bleeding after cut wound.There was a positive family history in 13 (65%) patients with haemophilia A and in 80% of haemophilia B. Among the haemophilia A patients, factor deficiency was severe in (75%), moderate in 3 patients and mild in 2 patients. In haemophilia B, 60% were moderate while 40% were severe.Most of the severe patients are on prophylactic treatment with no more evidence of target joints or severe bleeding. Only 3 patients developed high titer inhibitors for factor VIII. They are treated with bypassing agents. None of our patients developed viral infections from the plasma-derived product.ConclusionMost paediatric haemophilia patients in Kuwait have haemophilia A, which presents commonly with prolonged bleeding while haemophilia B (seen in 30%) is more likely to have mucous membrane bleeding.