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Bleeding after dental extraction was controlled with tranexamic acid in 19 patients with haemophilia and 3 with Christmas disease. The results were slightly better than those obtained with aminocaproic acid; the dose used was smaller; and side effects were few.
A 4-month-old boy presented with an apparently spontaneous uniocular hyphema as the initial manifestation of Christmas disease (hemophilia B). Although it is uncommon for patients with hemophilia to experience major bleeds in infancy, and the ocular involvement is usually limited to periocular hemorrhages, we recommend that a coagulation profile be obtained in all patients with intraocular hemorrhage to rule out this disease.
Serum creatine kinase, lactate dehydrogenase, aspartate and alanine transaminases, and aldolase were determined in 41 hospital inpatients with haemophilia or Christmas disease and no significant differences from the normal ranges were found.(3) Levels of these enzymes in a further 10 such patients who had sustained muscle haematomata were determined: in all of these there was a consistent rise in the level of creatine kinase, the peak occurring between 36 and 96 hours. In bleeding disorders a rise in serum creatine kinase levels may be useful as a diagnostic test for intramuscular haemorrhage.
A survey is presented of the use of the Oxford type DE(I) II-IX-X concentrate in the treatment of Christmas disease in Oxford from January 1970 to September 1974. 72 different patients were treated with a total of 2436 bottles of this concentrate from 143 different batches (each bottle containing 800-1000 units of factor IX). Although most doses were given for the treatment of minor haemorrhages into joints and muscles, 717 bottles of concentrate were used to treat 11 patients who underwent 14 major surgical operations. No episode of intravascular clotting or pulmonary embolism was seen in any patient receiving the concentrate. A detailed study of the plasma levels of factor V, VIII, total progressive antithrombin, platelets and fibrinogen degradation products was carried out before and after transfusion of type DE(I) concentrate in 14 patients. No significant alteration in those factors was found after the transfusion.
A I-year-old girl with severe Christmas disease and a factor IX content less than I% of normal is described. The family history was negative and coagulation studies on her relatives were normal. Genetic investigation showed an XXp-karyotype with deletion of the short arm of one X-chromosome, a cytogenetic variant of Turner syndrome. The transmission pathway of the haemophilia gene is discussed.
In a double-blind trial tranexamic acid (AMCA, Cyclokapron), 1 g three times a day for five days, significantly reduced blood loss and transfusion requirements after dental extraction in patients with haemophilia and Christmas disease. No side effects were seen in either group of patients. Screening tests showed no toxic action of tranexamic acid on the liver, kidney, or heart.
There has been no systematic re-examination of variables that may affect the level and duration of response of patients with hemophilia B (Christmas disease) to transfusion. Therefore, 49 of our transfusion episodes and 171 previously reported transfusions were evaluated. Mean calculated initial increase of Factor IX levels (delta %/unit (U) of procoagulant activity infused/kg) was 0.82 +/- 0.09% (mean +/-S.E.) in previously reported cases and 1.01 +/- 0.13% in our patients, after transfusion of concentrate; but only 0.05 +/- 0.11% after fresh frozen plasma (FFP). Response was not altered by acute hemorrhage, baseline Factor IX levels, or body weight. Proplex (Hyland) and Konyne (Cutter) produced similar responses. Following transfusion, the disappearance curve was biphasic. The mean T1/2 for the second component was 27.5 hrs, but the direct T1/2 was only 6.4 +/- 1.0 hr. Regardless of common clinical variables, increase of Factor IX following transfusion of American concentrates is 1.0% (or 0.01 U)/1 administered/kg. Appropriate frequency of transfusion depends upon an understanding of the biphasic disappearance of Factor IX. Importantly, the initial frequency of transfusion therapy should be based on a direct T1/2 of only 6 to 8 hrs.
A double-blind controlled study was carried out to investigate the effectiveness of treatment with factor VIII or factor IX concentrate and a reducing dose of prednisolone in contolling haematuria in patients with haemophilia and Christmas disease. 41 episodes of haematuria were studied in 30 different patients. No appreciable benefit was observed in the treated, as compared with the control group and this is at variance with the results of the few studies reported elsewhere.
Two cases of haemorrhagic diathesis are described, where the laboratory diagnosis was confused owing to previous blood transfusions. Without the help of investigating the relatives, the nephew of Case 1, the haemophiliac, and the uncle of Case 2, with Christmas disease, there would have been considerable delay in reaching the diagnosis and so instituting the correct treatment.
Evidence has been sought of activation of the coagulation system in two groups of patients following the infusion of two heparinised clotting factor concentrates. No changes were detected in 13 patients with mild hepatic dysfunction. In six studies on patients with Christmas disease induced abnormalities occurred in only one. Activation of the coagulation mechanism did not occur in another individual who had received the same batch of material.
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