Morbidity and mortality in a large number of Iranian patients with severe congenital factor XIII deficiency.

With 473 patients, Iran has about one third of the world's patients with severe congenital factor XIII (FXIII) deficiency. A considerable number of patients with FXIII deficiency (FXIIID) are affected by life-threatening bleeding episodes, such as central nervous system (CNS) bleeding or recurrent miscarriage and umbilical cord bleeding (UCB), that cause a high rate of morbidity and mortality in Iranian patients with FXIIID. Among 317 Iranian patients with FXIIID, 145 cases experienced 166 CNS bleeds (CNSBs) that recurred in 21 cases. CNSB caused different types of neurological complications in 69 patients. A total of 62 miscarriages were observed in 24 women of childbearing age, and 21 deaths were observed due to umbilical cord bleeding or mucosal bleeding. In fact, 49 deaths (15.4 %) were observed in these patients, which highlight the importance of early diagnosis and intensive health care among patients with FXIIID.

Reduced levels of coagulation factor XIII in patients with advanced tumor disease.

BACKGROUND/AIMS: Coagulation factor XIII, which induces the stabilization of fibrin the final step in the coagulation cascade, has various physiological effects. Among these, its beneficial effect in gastrointestinal bleeding episodes is well known. With the exception of inflammatory bowel disease, however, few data are available about this effect, particularly with regard to its role in diffuse bleeding in tumor patients. The study was designed to carry out prospective follow-up investigations, gathering data concerning factor XIII levels in patients with advanced gastrointestinal tumors and evaluating the course of the disease as well as the incidence of bleeding. METHODOLOGY: Sixty patients (22 women, 38 men; median age: 60; range: 29-79) with advanced gastrointestinal tumors were followed-up prospectively. Factor XIII levels were measured using chromogenic substrate. The correlation between the FXIII level and the patients' survival was analyzed using the Cox model. RESULTS: Factor XIII deficiency (below 70%) was seen in only 7 patients (11.6%), 6 of whom died within a median of 1.5 months after the measurement. In all patients however, there was a significant correlation (P = 0.0133) between FXIII levels and the risk of death. Four bleeding episodes occurred in 3 patients, three times with FXIII levels being below the lower normal range. When substitution was attempted, it was only successful in 1 patient in whom the FXIII level was reduced. CONCLUSIONS: FXIII may have predictive value as a marker for the prognosis in these patients with advanced tumor disease. Bleeding episodes were rarely seen, but when they do occur they may be associated with reduced levels of FXIII, and substitution may be beneficial as an adjunct or even as the sole therapeutic intervention.