Acquired Factor XIII inhibitor associated with mantle cell lymphoma: ACQUIRED FXIII INHIBITOR. Nixon, C. P., Prsic, E. H., Guertin, C. A., Stevenson, R. L., & Sweeney, J. D. Transfusion, 57(3):694–699, March, 2017. Number: 3![Acquired Factor XIII inhibitor associated with mantle cell lymphoma: ACQUIRED FXIII INHIBITOR [link]](https://bibbase.org/img/filetypes/link.svg "link")
Paper doi abstract bibtex 1 download BACKGROUND Acquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management. CASE REPORT A 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma. Initial management included an axillary lymph node biopsy and coagulation evaluation. Pathologic examination of the biopsy specimen revealed mantle cell lymphoma. Clot solubility assay was consistent with a FXIII activity of less than 3%. An anti-FXIII inhibitor was suspected, the epitope specificity of which was mapped by micropeptide array analysis to regions in the β-sandwich and catalytic core domain of the FXIII-A subunit. Management with cryoprecipitate, steroids, rituximab, and antifibrinolytic therapy resolved the bleeding diathesis and suppressed the inhibitor. CONCLUSION This is the first reported case of an acquired FXIII inhibitor associated with mantle cell lymphoma in which the epitope specificity of the pathologic autoantibody was accurately defined. Antifibrinolytic therapy played a prominent role in the prevention of bleeding complications in the window period between initiation of immunosuppression and disappearance of the pathologic anti-FXIII autoantibody.
@article{nixon_acquired_2017,
title = {Acquired {Factor} {XIII} inhibitor associated with mantle cell lymphoma: {ACQUIRED} {FXIII} {INHIBITOR}},
volume = {57},
issn = {00411132},
shorttitle = {Acquired {Factor} {XIII} inhibitor associated with mantle cell lymphoma},
url = {http://doi.wiley.com/10.1111/trf.13947},
doi = {10.1111/trf.13947},
abstract = {BACKGROUND
Acquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management.
CASE REPORT
A 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma. Initial management included an axillary lymph node biopsy and coagulation evaluation. Pathologic examination of the biopsy specimen revealed mantle cell lymphoma. Clot solubility assay was consistent with a FXIII activity of less than 3\%. An anti-FXIII inhibitor was suspected, the epitope specificity of which was mapped by micropeptide array analysis to regions in the β-sandwich and catalytic core domain of the FXIII-A subunit. Management with cryoprecipitate, steroids, rituximab, and antifibrinolytic therapy resolved the bleeding diathesis and suppressed the inhibitor.
CONCLUSION
This is the first reported case of an acquired FXIII inhibitor associated with mantle cell lymphoma in which the epitope specificity of the pathologic autoantibody was accurately defined. Antifibrinolytic therapy played a prominent role in the prevention of bleeding complications in the window period between initiation of immunosuppression and disappearance of the pathologic anti-FXIII autoantibody.},
language = {en},
number = {3},
urldate = {2019-11-28},
journal = {Transfusion},
author = {Nixon, Christian P. and Prsic, Elizabeth H. and Guertin, Christine A. and Stevenson, Ryan L. and Sweeney, Joseph D.},
month = mar,
year = {2017},
note = {Number: 3},
keywords = {Application - Antibody Validation / Epitope Mapping, Application - Autoimmune Research, Application - Cancer Research, Application - Other Disease, Country - USA, Human, PEPperCHIP - Customized - Linear, PEPperMAP - Epitope Mapping - Linear, Sample Type - Plasma},
pages = {694--699},
}
Downloads: 1
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CASE REPORT A 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma. Initial management included an axillary lymph node biopsy and coagulation evaluation. Pathologic examination of the biopsy specimen revealed mantle cell lymphoma. Clot solubility assay was consistent with a FXIII activity of less than 3%. An anti-FXIII inhibitor was suspected, the epitope specificity of which was mapped by micropeptide array analysis to regions in the β-sandwich and catalytic core domain of the FXIII-A subunit. Management with cryoprecipitate, steroids, rituximab, and antifibrinolytic therapy resolved the bleeding diathesis and suppressed the inhibitor. CONCLUSION This is the first reported case of an acquired FXIII inhibitor associated with mantle cell lymphoma in which the epitope specificity of the pathologic autoantibody was accurately defined. Antifibrinolytic therapy played a prominent role in the prevention of bleeding complications in the window period between initiation of immunosuppression and disappearance of the pathologic anti-FXIII autoantibody.","language":"en","number":"3","urldate":"2019-11-28","journal":"Transfusion","author":[{"propositions":[],"lastnames":["Nixon"],"firstnames":["Christian","P."],"suffixes":[]},{"propositions":[],"lastnames":["Prsic"],"firstnames":["Elizabeth","H."],"suffixes":[]},{"propositions":[],"lastnames":["Guertin"],"firstnames":["Christine","A."],"suffixes":[]},{"propositions":[],"lastnames":["Stevenson"],"firstnames":["Ryan","L."],"suffixes":[]},{"propositions":[],"lastnames":["Sweeney"],"firstnames":["Joseph","D."],"suffixes":[]}],"month":"March","year":"2017","note":"Number: 3","keywords":"Application - Antibody Validation / Epitope Mapping, Application - Autoimmune Research, Application - Cancer Research, Application - Other Disease, Country - USA, Human, PEPperCHIP - Customized - Linear, PEPperMAP - Epitope Mapping - Linear, Sample Type - Plasma","pages":"694–699","bibtex":"@article{nixon_acquired_2017,\n\ttitle = {Acquired {Factor} {XIII} inhibitor associated with mantle cell lymphoma: {ACQUIRED} {FXIII} {INHIBITOR}},\n\tvolume = {57},\n\tissn = {00411132},\n\tshorttitle = {Acquired {Factor} {XIII} inhibitor associated with mantle cell lymphoma},\n\turl = {http://doi.wiley.com/10.1111/trf.13947},\n\tdoi = {10.1111/trf.13947},\n\tabstract = {BACKGROUND\nAcquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management.\n\nCASE REPORT\nA 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma. Initial management included an axillary lymph node biopsy and coagulation evaluation. Pathologic examination of the biopsy specimen revealed mantle cell lymphoma. Clot solubility assay was consistent with a FXIII activity of less than 3\\%. An anti-FXIII inhibitor was suspected, the epitope specificity of which was mapped by micropeptide array analysis to regions in the β-sandwich and catalytic core domain of the FXIII-A subunit. Management with cryoprecipitate, steroids, rituximab, and antifibrinolytic therapy resolved the bleeding diathesis and suppressed the inhibitor.\n\nCONCLUSION\nThis is the first reported case of an acquired FXIII inhibitor associated with mantle cell lymphoma in which the epitope specificity of the pathologic autoantibody was accurately defined. 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