B. Pollio, P. Saracco, I. Ricca, T. Ceglie, C. Linari, R. Albiani
A Pakistani boy with an inherited FXIII deficiency came at our attention with a suspect of compartment syndrome, and was successfully treated with recombinant r-FXIII-A replacement. We would like to emphasize the efficacy of rFXIII-A, compared with fresh frozen plasma and cryoprecipitates, which did not cause a significant increase in FXIII levels in our patient. In 2017, our experience had already showed that Catridecacog could be used safely and effectively not only for continued prophylaxis but also for on-demand treatment and adds to the limited body of evidence currently available on rFXIII-A for acute bleedings.
Factor XIII (FXIII) is a protransglutaminase that, after activation by thrombin and in the presence of calcium, becomes transglutaminase, leading to increased stability of the fibrin clot. Plasma FXIII is a heterotetramer composed of 2 catalytic A-subunit and 2 carrier Bsubunits linked by noncovalent bonds. Inherited FXIII deficiency is a rare bleeding disorder caused by defects in both FXIIIA and FXIIIB genes; however, the majority of the cases are attributed to genetic variants of the FXIIIA gene...