International journal of hematology
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Factor XIII (FXIII) deficiency is a rare hemorrhagic disorder for which the highest incidence occurs in southeast Iran. The aim of this study was to assess molecular characteristics, clinical manifestations and management of life-threatening diathesis in FXIII deficiency. This study was conducted on 190 patients with FXIII deficiency. ⋯ Neonates of group 2 received 60-80 IU/kg dose of Fibrogammin P(®). This higher dose did not trigger thrombotic events but significantly decreased bleeding episodes and prevented the occurrence of major bleeding. Trp187Arg is the most common mutation of FXIII-A subunit in Iran, and Fibrogammin P(®) is effective in the management of FXIII deficiency, and higher dose of this drug is safe and effective in neonates.