Page 2170 - Williams Hematology ( PDFDrive )

2144 Part XII: Hemostasis and Thrombosis Chapter 124: Inherited Deficiencies of Coagulation Factors II, V, V+VIII, VII, X, XI, and XIII 2145

No large clinical reports on heterozygous patients with factor XIII pulmonary embolism, Crohn disease, ulcerative colitis, Henoch-Schön-
deficiency are available, thus not allowing to draw evidence-based con- lein purpura, liver cirrhosis, and sepsis. There are several case reports of
clusion on the prevalence of clinical symptoms in this group of patients. an autoimmune bleeding disorder, designated as autoimmune/acquired
Recently, a subset of 28 heterozygotes for factor XIII deficiency among hemorrhaphilia, being caused by anti–factor XIII inhibitors. The anti–
328
350 carriers of an autosomal recessive inherited coagulation disorders factor XIII inhibitors tend to be more severe than regular hemorrhagic-
show an association with prolonged or massive bleeding after minor acquired factor XIII deficiency and requires both immunosuppressive
310
trauma. However, these data need to be confirmed in other cohorts therapy to eradicate autoantibodies and factor XIII replacement therapy
of patients. to stop the bleeding. 329
Factor XIII A-subunit knockout mice manifest no excess embry-
onic lethality or bleeding into the thoracic cavity, peritoneum, or skin,
compatible with survival to adulthood. However, the survival rate of REFERENCES
311
knockout males was markedly lower than that of the wild-types.
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