Acquired factor XIII deficiency in myeloid neoplasms: case series and review of literature.

Abstract

Acquired factor XIII (FXIII) deficiency is a rare disorder that could be associated with autoimmune and malignant disorders with a high risk of bleeding. In acute leukemias, acquired FXIII deficiency has been reported and replacement of FXIII helped to control significant bleeding. Here, we report four cases of myeloid neoplasms to have acquired FXIII deficiency with interesting concomitant RUNX1 mutation in the molecular background of two patients. Correction of bleeding complications was successful in all patients with FXIII substitution by FXIII concentrate in three patients and cryoprecipitate in one patient. Studying the association of FXIII deficiency with molecular abnormalities in such neoplasms is needed for better understanding and detection of common pathophysiologic pathways. This could help to avoid severe bleeding during diagnostic or therapeutic interventions in patients at risk.