Factor XIII Deficiency

Classification according to ICD-10
D68.2	Hereditary deficiency of other coagulation factors - deficiency of factor XIII (fibrin stabilizing factor)
ICD-10 online (WHO version 2019)

The Factor XIII deficiency is a very rare disorder of hemostasis due to an autosomal - recessive inherited defect on fibrin-stabilizing factor (Factor XIII). The affected person has less than one percent of the normal amount of the factor.

Symptoms

In homozygous newborns , massive umbilical cord bleeding often occurs (in up to 80 percent of births) . In homozygous adults there are wound healing disorders , increased cerebral haemorrhage and secondary bleeding. Also miscarriages are heaped. Oligospermia and shrinkage of the testicles have been described in male patients .

Heterozygous people are asymptomatic .

therapy

A factor XIII concentrate is given prophylactically for therapy . Such concentrates are obtained from blood plasma by plasma fractionation . For a number of years there have been preparations produced in vitro.

literature

• CS Kitchens, TF Newcomb: Factor XIII. In: Medicine (Baltimore). 1979 Nov; 58 (6), pp. 413-429. PMID 514066

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !