Hemophilic Arthropaty: A Case Report / 대한정형외과학회잡지

ABSTRACT

Hemophilia is a sex-linked recessive hereditary bleeding disorder occurring only in the male and transmitted by the female. This disease is characterized by a bleeding tendency due to prolonged coagulation time causing by deficiency of one of three plasma factors, such as anti-hemophilie globulin(A. H. G.), plasma thromboplastin component(P. T. C.) and plasma thromboplastin antecedent (P. T. A.) for first phase of coagulation process. The majority of hemophilia, 74% is due to a deficiency of A. H. G., 15% to a deficiency of P. T. C. and remaining 11% to a deficiency of P. T. A. as outlined in the literature. This case, 18 years old male, is complained of repeated hemorrhagic manifestations, residual deformity with stiffness of the hip and knee joints, and limping following minor trauma has developed since early childhood. Radiologically, the hip and knee joints were involved, showing the findings of typical hemophilic arthropathy. Laboratory finding showed markedly prolonged coagulation time. Diagnosis was confirmed to be plasma thromboplastin antecedent deficiency homophilia.