Acquired Factor XIII inhibitor associated with mantle cell lymphoma

• DOI: 10.1111/trf.13947
• Publication Date: 2016-12-05T03:10:39Z
• Authors: Christian P. Nixon; Elizabeth H. Prsic; Christine A. Guertin; Ryan L. Stevenson; Joseph D. Sweeney
• Keywords: Antifibrinolytic; Bleeding diathesis; Hyperfibrinolysis

BACKGROUND Acquired Factor (F)XIII deficiency is a very rare bleeding diathesis with potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There minimal information on autoantibody characterization role antifibrinolytic therapy patient management. CASE REPORT A 79‐year‐old woman 3‐month history bruising heavy menorrhagia presented ongoing vaginal bleeding, symptomatic anemia, right thigh hematoma. Initial management included an axillary lymph node biopsy coagulation evaluation. Pathologic examination specimen revealed mantle cell lymphoma. Clot solubility assay was consistent FXIII activity less than 3%. An anti‐FXIII inhibitor suspected, epitope specificity which mapped by micropeptide array analysis to regions β‐sandwich catalytic core domain FXIII‐A subunit. Management cryoprecipitate, steroids, rituximab, resolved suppressed inhibitor. CONCLUSION This first reported case acquired associated lymphoma pathologic accurately defined. Antifibrinolytic played prominent prevention complications window period between initiation immunosuppression disappearance autoantibody.