The author reviewed 15 cases of hemophilia seen at Kyungpook Univeristy Hospital during the 10 year period, 1958-1967, with the following results.
1) Thirteen of the total 15 patients were AHG deficiency, one was PTC deficiency and the remaining one type was undetermined.
2) All were male and in approximately 40% of them, familial antecedents could be demonstrated.
3) An initial bleeding episode occurred in all cases before they reached the age of five and in two thirds of the cases occurred in the first year of life.
4) Hematoma was the earliest hemorrhagic manifestation and when they reached the age of active ambulation, hemarthrosis became the most prominent hemorrhagic symptom.
5) A coagulation study revealed platelet count, capillary fragility, bleeding time, clot retraction, prothrombin time, plasma thrombin time and tests to be normal. However, coagulation time as well as bleeding time were markedly prolonged and prothrombin consumption poor. Fibrin stabilizing factor was found to be decreased in two of three patients studied.
6) Prothrombin consumption test was employed for hemophilia type determination. An abnormality in blood with AGH deficiency was corrected by BaSO₄absorbed plasma but not by old serum. On the contrary, in PTC deficiency the abnormality was corrected by old serum but not by BaSO₄absorbed plasma.
7) Patients were treated successfully with fresh whole blood for hemostasis. One patient had been given epsilon aminocaproic acid for two years with decreased episodes of hemarthrosis.
|