What is Hereditary Factor X Deficiency?
The existence of factor X and its hereditary deficiency was first proved by the scientist Telfer, as well as the co-authors in 1956
Stewart’s disease – Prouer – a rare disease, inherited by an incomplete autosomal recessive type.
Symptoms of Hereditary Factor X Deficiency
Both very severe forms (in homozygotes) and light and latent varieties (in heterozygotes) are possible. With very severe forms, the concentration of factor X in plasma does not reach 1%, with severe it is within 1-2%, moderately severe – from 2 to 5%, with light – from 5 to 10%, with latent – 10% and higher. The concentration of factor X, equal to 10%, is considered to be minimal enough for proper hemostasis.
In cases where the amount of factor X cannot be determined, a tentative judgment about its deficiency can be made by lengthening the prothrombin time: when the level of factor X is less than 1%, this indicator exceeds 90 s, at a level from 1 to 2% is 70–90 s, from 2 to 5% – 40-70 s, from 5 to 10% – 15-40 s (the norm is 12-14 s).
When a very severe form of the disease bleeding occurs in early childhood, often already at birth or in the first months of life, often causing deaths. Recurrent hemorrhages in the brain and abundant gastrointestinal bleeding are the immediate cause of death.
In severe forms of the disease, bleeding occurs somewhat later and does not become a disaster. Small hemorrhages in the skin, subcutaneous hemorrhages, abundant and prolonged nasal and gingival bleeding, and especially painful uterine bleeding in patients, are quite frequent.
Surgical interventions, childbirth and abortion are accompanied by copious, life-threatening bleeding. Intramuscular hemorrhages and hemorrhages in the joints are extremely rare. In the moderate form of the disease, uterine, nasal and gingival bleeding, hemorrhages into the skin, bleeding from injuries and operations are most pronounced. The mild form does not give such severe symptoms, but still quite pronounced menstrual bleeding, bleeding during childbirth and during surgical interventions. Periodically, there are “causeless” nosebleeds. Mild disease has long light periods without any bleeding.
Diagnosis of Inherited Factor X Deficiency
A prolongation of prothrombin time is characteristic of Stuart-Prouer’s disease. The second typical feature of factor X deficiency is the elongation of the kaolin-kefalin coagulation time, a decrease in the consumption of prothrombin.
However, in milder forms of the Stewart-Prouwer disease with a concentration of factor X above 7-8%, tests of the internal coagulation mechanism may remain almost normal. When evaluating the testimony of laboratory tests, you should always consider the severity of the disease and the lengthening of the prothrombin time.
Of considerable importance in the diagnosis of deficiency of factors X and VII are samples with snake venoms.
Treatment of Inherited Factor X Deficiency
Transfusion therapy of Stewart’s disease – Prauer is carried out with the same hemopreparations and in the same doses as with replacement therapy for factor VII deficiency.
Plasma transfusions with factor X deficiency are carried out once a day. Transfusion is indicated for all types of bleeding, for surgical interventions (most often from the 1st to the 10th day after the operation), during childbirth and in the first 5 days of the postpartum period.
Simultaneous administration of progestins and PPSB should be avoided because of the danger of disseminated intravascular coagulation.
Locally for nosebleeds, bleeding from the tooth holes, hemostatic sponge, 5-10% solution of aminocaproic acid is used quite effectively.