MILD HÆMOPHILIA‐A CLINICAL AND LABORATORY STUDY

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Summary: In a series of 65 patients suffering from hæmophilia (AHG deficiency), 19 (29%) were found to have a normal whole‐blood coagulation time. These patients suffered from a mild form of the disease as assessed clinically and on the results of laboratory tests. Ages ranged from nine to sixty‐seven years. Seventeen families were involved; in eight of these other members were affected.Hæmorrhagic episodes were mostly related to periods when the hæmostatic mechanism was placed under stress, as after tooth extraction or surgical procedures. Spontaneous hæmorrhages and hæmarthroses were uncommon symptoms.The whole blood coagulation time was normal in all 19 patients. The prothrombin consumption index was normal (0% to 40%) in 13 patients and borderline (20% to 40%) in seven. The result of the thrombin generation test was normal in to of 15 patients tested. The most reliable method for diagnosis was the thromboplastin‐generation test. This test gave abnormal findings in 15 patients, and with a modification involving use of a greater dilution of plasma gave an abnormal result in all patients.Plasma AHG concentrations have been measured in these patients, and the results confirm that at least 30% AHG is necessary for normal hæmostasis.
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