Factor 13 deficiency with severe hemorrhagic diathesis.

A HEMORRHAGIC DIATHESIS due to a congenital deficiency of fibrin stabilizing factor (f.s.f.) was first described in 1960 by Duckert et al.1 Five other cases were discovered since, in different cOuntries.2'6 The fibrin stabilizing factor (Lorand-Laki factor, fibrinase of Loewy), known and investigated in vitro for almost 20 years,7-11 was accepted in 1963 as one of the clotting factors-i.e. Factor 13. This factor determines enzy-maticalby a strong stable linkage between the fibrin monomers of the blood clots. In its absence, the fibrin polymer is weak, its molecules are aggregated by hydrogen bonds only, and consequently are soluble, in vitro, in concentrated solutions of urea. Here we report the first case of Factor 13 deficiency in an adult female who exhibited various bleeding manifestations and whose pregnancies were all interrupted by spontaneous abortions due to severe decidual hemorrhage.'2 By treating the clotting defect, her last pregnancy proceeded normally without any bleeding, and she gave birth to a normal child. CASE REPORT R. B. S., a 29-year-old woman born in Morocco, married and childless, was referred to the hematologic clinic in June 1963 by the gynecologic departmiient of our hospital. because of hemorrhagic diathesis with repeated abortions. She had been admitted on several occasions to the gynecological department (Dr. Rikover, Dr. Naor) because of these abortions. The bleeding history started at birth, with a severe and prolonged umbilical vein hemorrhage after the ligation of the umbilical cord. Since childhood she had repeatedly suffered severe bleeding after cuts, or dental extractions and hematoma into the skin after minimal trauma. She also exhibited slow healing of wounds. In the last 10 years. the patient had been admitted 4 times to various hospitals because of Douglas hematoma with severe abdominal and perineal pains and at times urinary disorders. In 1957 she had been hospitalized for 6 months, with what was finally diagnosed as a large pelvic hematoma with transient neurologic disorders due to compression. In 1960 she had an episode of painless hematuria. During these episodes the patient was umsumally treated with blood transfusions. The patient's menstrual periods were normal. Bumt, most important, all her 12 pregnancies were interrupted at various stages of fetal development by spontaneous abortions with severe bleedings. No gynecologic or other disorders could be found as an explanation to the repeated abortions. The physical and gvnecologic examinations were negative. The patient was a child of a consanguineoums marriage: the father was …