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Gemetoc aspects of Factor XIII deficiency

Factor XI11 (fibrin stabilizing factor) occurs in plasma as a proenzyme which, after activation, participates in the final stage of the blood coagulation process. Specifically, it catalyses the formation of y-glutamyl-e-lysyl bonds in fibrin. Fibrin stabilized in this manner has greater elasticity (Lorand, 1970) and is more resistant to fibrinolysis (McDonagh, McDonagh & Duckert, 1971) than fibrin which has not been stabilized by Factor XIII. Stabilized fibrin is also insoluble in urea or dilute acids, whereas fibrin which has not been acted upon by Factor XI11 is readily soluble in these solvents. Haeniorrhagic diathesis due to congenital deficiency of Factor XI11 is usually characterized by umbilical cord bleeding as the first symptom and a high incidence of haematomata and ecchymoses following mild trauma. Spontaneous haemarthroses, intracranial haemorrhage, and decidual bleeding with spontaneous abortion of pregnancy have also been observed. Diagnosis of a patient with Factor XI11 deficiency can be confirmed in the laboratory by the rapid solubility of the patient’s recalcified plasma clot in 5 M molar urea or 1 % monochloroacetic acid. Since the report of Duckert, Jung & Shmerling (1960) of the first case of congenital Factor XI11 deficiency, many such cases have been documented in the literature (see Table 2). It is generally agreed that, like many other coagulopathies, congenital deficiency of Factor XI11 is a hereditary disorder; but the mode of inheritance is still in question. It has been suggested by various authors that transmission of Factor XI11 deficiency is autosomal recessive (Ikkala, 1968) or X-linked recessive (Hampton, Cunningham & Bird, 1966) or that there are two mcchanisms of transmission, one which is autosomal recessive and one which is X-linked recessive (Ratnoff & Steinberg, 1968). In this study the family of the first patient described by Duckert et al. was reinvestigated with a sensitive new assay for Factor XI11 in order to study further the pattern of inheritance and the detection of heterozygotes.