Factor XIII Deficiency: A Review of Clinical Presentation and Management

Authors: Pelcovits, A; Schiffman, F; Niroula, R.

Affiliations: Alpert Medical School of Brown University, Providence, RI, USA; Division of Hematology-Oncology, Rhode Island Hospital and The Miriam Hospital, 164 Summit Avenue, Providence, RI 02906, USA.

Publication: Hematology/oncology Clinics of North America. 2021

ABSTRACT: Factor XIII (FXIII) deficiency is a rare autosomal recessive disorder that can result in life-threatening bleeding and early fetal loss. FXIII not only is responsible for cross-linking fibrinogen to stabilize and strengthen clot formation but also facilitates wound healing and angiogenesis and plays an important role in fetal vitality. Modern therapeutics allow for prophylactic treatment that can prevent most major bleeding and increasing fetal viability. Early diagnosis is paramount due to the high risk of intracranial bleeding.