Intracranial hemorrhage pattern in the patients with factor XIII deficiency

Abstract

Intracranial hemorrhage (ICH) is one of the most severe and life-threatening manifestations occurring in the patients with factor XIII (F XIII) deficiency. The aim of this study was to describe the ICH pattern in the patients suffering from F XIII deficiency. In this case series, we investigated 38 patients with severe F XIII deficiency in south of Iran from January to May 2012. ICH pattern, neurologic complications, efficacy of treatment, and incidence of recurrence were reported. The site of ICH was intraparenchymal in 35 patients (92.1 %), subdural in 2 patients (5.2 %), and epidural hemorrhage in 1 patient (2.6 %). Besides, neurologic complications occurred in 21 patients (55.2 %), including locomotor disability in 8, psychological impairment in 7, mental disorders in 5, speech impairment in 4, and visual impairment in 2. Prophylaxis was started with a dose of 10 IU/kg Fibrogammin every 4-6 weeks for all the patients, except for one. All the patients on prophylaxis showed good response without any episodes of recurrence, except for one. The most frequent site of ICH in our patients was intraparenchymal. It seems that long-term prophylactic treatment with a dose of 10 IU/kg Fibrogammin could be effective in the prevention of CNS bleeding in the patients with F XIII deficiency. Moreover, all the patients with severe F XIII deficiency even without severe bleeding symptoms are recommended to undergo prophylactic treatment.

Establishment of a prenatal diagnosis schedule as part of a prophylaxis program of factor XIII deficiency in the southeast of Iran

“…We previously reported this high rate of morbidity and mortality among patients with FXIIID, among whom CNS bleeding (83%) was the most common cause of mortality [8]. Moreover, CNS bleeding in the majority of the affected individuals causes neurologic complications, including locomotor disability, psychological impairment, mental disorders, as well as speech and visual impairment [10]. Another important finding was genetic predisposition to CNS bleeding among our patients with FXIIID.…”

Factor XIII deficiency revealed by spontaneous intramedullary hemorrhage

“…When congenital, it presents with severe bleeding, neonatal umbilical bleeding, spontaneous intracranial hemorrhages, poor wound healing, and spontaneous abortions. More commonly, FXIII deficiency is acquired with certain drugs or autoimmune disorders by developing an autoantibody binding to FXIII and interfering with its normal function [1,4]. The results of standard laboratory clotting tests are all normal in FXIII-deficient patients.…”

Epidemiology of Hereditary Coagulation Bleeding Disorders: A 15-Year Experience From Southern Iran

“…In addition, ICH has also been reported to be higher than the normal population in patients with factor X, factor V, factor VII, and factor XIII deficiencies. 17 Khleif et al found the prevalence of neurological diseases including seizure, Parkinson disease, and dementia to be 5%. They also found the prevalence of respiratory diseases, including asthma, to be 11% among these patients.…”