Limited value of testing for factor XIII and α2‐antiplasmin deficiency in patients with a bleeding disorder of unknown cause
Introduction In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2‐ant...
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| Veröffentlicht in: | Haemophilia : the official journal of the World Federation of Hemophilia 2024-07, Vol.30 (4), p.998-1002 |
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| container_title | Haemophilia : the official journal of the World Federation of Hemophilia |
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| creator | Ariëns, Sander Huisman, Albert Hovinga, Idske C. L. Kremer Urbanus, Rolf T. Galen, Karin P. M. Vulpen, Lize F. D. Fischer, Kathelijn Schutgens, Roger E. G. |
| description | Introduction
In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2‐antiplasmin (α2AP) deficiency.
Aim
To evaluate the added diagnostic value of FXIII and α2AP levels in patients with a bleeding disorder of unknown cause (BDUC).
Methods
A retrospective monocentre cohort study between August 2011 and August 2023 was conducted. In all patients with bleeding tendencies and normal diagnostic tests for von Willebrand disease and platelet function, FXIII and α2AP were measured.
Results
We included 158 consecutive patients; mean ISTH‐BAT scores were 8.2 (SD ± 3.7) in children, 6.2 (SD ± 2.1) in men and 10.6 (SD ± 3.3) in women. Median age was 37 (range 5–79) years, 88.6% of patients were female. Patients displayed median FXIII activity of 111% (IQR = 97–131) and median α2AP activity of 112% (IQR = 103–119).
Three (1.9%) patients had FXIII levels |
| doi_str_mv | 10.1111/hae.15059 |
| format | Article |
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In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2‐antiplasmin (α2AP) deficiency.
Aim
To evaluate the added diagnostic value of FXIII and α2AP levels in patients with a bleeding disorder of unknown cause (BDUC).
Methods
A retrospective monocentre cohort study between August 2011 and August 2023 was conducted. In all patients with bleeding tendencies and normal diagnostic tests for von Willebrand disease and platelet function, FXIII and α2AP were measured.
Results
We included 158 consecutive patients; mean ISTH‐BAT scores were 8.2 (SD ± 3.7) in children, 6.2 (SD ± 2.1) in men and 10.6 (SD ± 3.3) in women. Median age was 37 (range 5–79) years, 88.6% of patients were female. Patients displayed median FXIII activity of 111% (IQR = 97–131) and median α2AP activity of 112% (IQR = 103–119).
Three (1.9%) patients had FXIII levels < 50%, respectively 43%, 45% and 46%. Corresponding ISTH‐BAT scores were 7, 12 and 14. No α2AP levels < 60% was observed. No significant association was found between FXIII levels and ISTH‐BAT scores.
Conclusion
In our cohort of BDUC patients, no clinical relevant FXIII deficiencies were detected; absolute values were well above the 30% cutoff considered adequate for normal haemostasis. No α2AP deficiencies were detected. These data suggest that in BDUC patients, measuring FXIII or AP activity is of limited value.</description><identifier>ISSN: 1351-8216</identifier><identifier>ISSN: 1365-2516</identifier><identifier>EISSN: 1365-2516</identifier><identifier>DOI: 10.1111/hae.15059</identifier><identifier>PMID: 38812123</identifier><language>eng</language><publisher>England: Wiley Subscription Services, Inc</publisher><subject>alpha‐2‐antiplasmin ; Bleeding ; bleeding disorder of unknown cause (BDUC) ; Blood coagulation ; Coagulation factor XIII ; Coagulation factors ; factor XIII deficiency ; haemorrhagic disorders ; Hematological diseases ; primary haemostasis</subject><ispartof>Haemophilia : the official journal of the World Federation of Hemophilia, 2024-07, Vol.30 (4), p.998-1002</ispartof><rights>2024 The Author(s). published by John Wiley & Sons Ltd.</rights><rights>2024 The Author(s). Haemophilia published by John Wiley & Sons Ltd.</rights><rights>2024. This article is published under http://creativecommons.org/licenses/by-nc-nd/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c2789-87ead8c6e8b45e9a1ef30e00c0acc3c49f024d81b85b06b2a32631ee719ac04b3</cites><orcidid>0000-0002-2762-6033 ; 0000-0003-3251-8595 ; 0009-0003-9675-122X ; 0000-0002-6244-0429 ; 0000-0001-7126-6613 ; 0000-0003-3242-5524</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fhae.15059$$EPDF$$P50$$Gwiley$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fhae.15059$$EHTML$$P50$$Gwiley$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38812123$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ariëns, Sander</creatorcontrib><creatorcontrib>Huisman, Albert</creatorcontrib><creatorcontrib>Hovinga, Idske C. L. Kremer</creatorcontrib><creatorcontrib>Urbanus, Rolf T.</creatorcontrib><creatorcontrib>Galen, Karin P. M.</creatorcontrib><creatorcontrib>Vulpen, Lize F. D.</creatorcontrib><creatorcontrib>Fischer, Kathelijn</creatorcontrib><creatorcontrib>Schutgens, Roger E. G.</creatorcontrib><title>Limited value of testing for factor XIII and α2‐antiplasmin deficiency in patients with a bleeding disorder of unknown cause</title><title>Haemophilia : the official journal of the World Federation of Hemophilia</title><addtitle>Haemophilia</addtitle><description>Introduction
In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2‐antiplasmin (α2AP) deficiency.
Aim
To evaluate the added diagnostic value of FXIII and α2AP levels in patients with a bleeding disorder of unknown cause (BDUC).
Methods
A retrospective monocentre cohort study between August 2011 and August 2023 was conducted. In all patients with bleeding tendencies and normal diagnostic tests for von Willebrand disease and platelet function, FXIII and α2AP were measured.
Results
We included 158 consecutive patients; mean ISTH‐BAT scores were 8.2 (SD ± 3.7) in children, 6.2 (SD ± 2.1) in men and 10.6 (SD ± 3.3) in women. Median age was 37 (range 5–79) years, 88.6% of patients were female. Patients displayed median FXIII activity of 111% (IQR = 97–131) and median α2AP activity of 112% (IQR = 103–119).
Three (1.9%) patients had FXIII levels < 50%, respectively 43%, 45% and 46%. Corresponding ISTH‐BAT scores were 7, 12 and 14. No α2AP levels < 60% was observed. No significant association was found between FXIII levels and ISTH‐BAT scores.
Conclusion
In our cohort of BDUC patients, no clinical relevant FXIII deficiencies were detected; absolute values were well above the 30% cutoff considered adequate for normal haemostasis. No α2AP deficiencies were detected. These data suggest that in BDUC patients, measuring FXIII or AP activity is of limited value.</description><subject>alpha‐2‐antiplasmin</subject><subject>Bleeding</subject><subject>bleeding disorder of unknown cause (BDUC)</subject><subject>Blood coagulation</subject><subject>Coagulation factor XIII</subject><subject>Coagulation factors</subject><subject>factor XIII deficiency</subject><subject>haemorrhagic disorders</subject><subject>Hematological diseases</subject><subject>primary haemostasis</subject><issn>1351-8216</issn><issn>1365-2516</issn><issn>1365-2516</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><recordid>eNp1kc9O20AQh1cIBCFw6AtUK3EpB4f9YzvrY4QoiRSJSyv1Zo13x82CvU537UY5wSP0VXgRHoIn6aaBHip1LzMjffp2Rj9CPnA24fFdrQAnPGNZcUBGXOZZIjKeH-76jCdK8PyEnIZwzxiXguXH5EQqxQUXckQel7a1PRr6E5oBaVfTHkNv3Xdad57WoPtYvi0WCwrO0Jdn8fr0C1xv1w2E1jpqsLbaotNbGqc19LHvA93YfkWBVg2i2cmMDZ036HcfDO7BdRtHNQwBz8hRDU3A87c6Jl8_33y5nifLu9vF9WyZaDFVRaKmCEbpHFWVZlgAx1oyZEwz0FrqtKiZSI3ilcoqllcCpMglR5zyAjRLKzkmn_bete9-DPHEsrVBY9OAw24IpWS5yKQQjEX04h_0vhu8i9tFSvFUpZGN1OWe0r4LwWNdrr1twW9LzspdKmVMpfyTSmQ_vhmHqkXzl3yPIQJXe2BjG9z-31TOZzd75W8NFJgz</recordid><startdate>202407</startdate><enddate>202407</enddate><creator>Ariëns, Sander</creator><creator>Huisman, Albert</creator><creator>Hovinga, Idske C. L. Kremer</creator><creator>Urbanus, Rolf T.</creator><creator>Galen, Karin P. M.</creator><creator>Vulpen, Lize F. D.</creator><creator>Fischer, Kathelijn</creator><creator>Schutgens, Roger E. G.</creator><general>Wiley Subscription Services, Inc</general><scope>24P</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>8FD</scope><scope>FR3</scope><scope>H94</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-2762-6033</orcidid><orcidid>https://orcid.org/0000-0003-3251-8595</orcidid><orcidid>https://orcid.org/0009-0003-9675-122X</orcidid><orcidid>https://orcid.org/0000-0002-6244-0429</orcidid><orcidid>https://orcid.org/0000-0001-7126-6613</orcidid><orcidid>https://orcid.org/0000-0003-3242-5524</orcidid></search><sort><creationdate>202407</creationdate><title>Limited value of testing for factor XIII and α2‐antiplasmin deficiency in patients with a bleeding disorder of unknown cause</title><author>Ariëns, Sander ; Huisman, Albert ; Hovinga, Idske C. L. Kremer ; Urbanus, Rolf T. ; Galen, Karin P. M. ; Vulpen, Lize F. D. ; Fischer, Kathelijn ; Schutgens, Roger E. G.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c2789-87ead8c6e8b45e9a1ef30e00c0acc3c49f024d81b85b06b2a32631ee719ac04b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>alpha‐2‐antiplasmin</topic><topic>Bleeding</topic><topic>bleeding disorder of unknown cause (BDUC)</topic><topic>Blood coagulation</topic><topic>Coagulation factor XIII</topic><topic>Coagulation factors</topic><topic>factor XIII deficiency</topic><topic>haemorrhagic disorders</topic><topic>Hematological diseases</topic><topic>primary haemostasis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ariëns, Sander</creatorcontrib><creatorcontrib>Huisman, Albert</creatorcontrib><creatorcontrib>Hovinga, Idske C. L. Kremer</creatorcontrib><creatorcontrib>Urbanus, Rolf T.</creatorcontrib><creatorcontrib>Galen, Karin P. M.</creatorcontrib><creatorcontrib>Vulpen, Lize F. D.</creatorcontrib><creatorcontrib>Fischer, Kathelijn</creatorcontrib><creatorcontrib>Schutgens, Roger E. G.</creatorcontrib><collection>Wiley Online Library Open Access</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Haemophilia : the official journal of the World Federation of Hemophilia</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ariëns, Sander</au><au>Huisman, Albert</au><au>Hovinga, Idske C. L. Kremer</au><au>Urbanus, Rolf T.</au><au>Galen, Karin P. M.</au><au>Vulpen, Lize F. D.</au><au>Fischer, Kathelijn</au><au>Schutgens, Roger E. G.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Limited value of testing for factor XIII and α2‐antiplasmin deficiency in patients with a bleeding disorder of unknown cause</atitle><jtitle>Haemophilia : the official journal of the World Federation of Hemophilia</jtitle><addtitle>Haemophilia</addtitle><date>2024-07</date><risdate>2024</risdate><volume>30</volume><issue>4</issue><spage>998</spage><epage>1002</epage><pages>998-1002</pages><issn>1351-8216</issn><issn>1365-2516</issn><eissn>1365-2516</eissn><abstract>Introduction
In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2‐antiplasmin (α2AP) deficiency.
Aim
To evaluate the added diagnostic value of FXIII and α2AP levels in patients with a bleeding disorder of unknown cause (BDUC).
Methods
A retrospective monocentre cohort study between August 2011 and August 2023 was conducted. In all patients with bleeding tendencies and normal diagnostic tests for von Willebrand disease and platelet function, FXIII and α2AP were measured.
Results
We included 158 consecutive patients; mean ISTH‐BAT scores were 8.2 (SD ± 3.7) in children, 6.2 (SD ± 2.1) in men and 10.6 (SD ± 3.3) in women. Median age was 37 (range 5–79) years, 88.6% of patients were female. Patients displayed median FXIII activity of 111% (IQR = 97–131) and median α2AP activity of 112% (IQR = 103–119).
Three (1.9%) patients had FXIII levels < 50%, respectively 43%, 45% and 46%. Corresponding ISTH‐BAT scores were 7, 12 and 14. No α2AP levels < 60% was observed. No significant association was found between FXIII levels and ISTH‐BAT scores.
Conclusion
In our cohort of BDUC patients, no clinical relevant FXIII deficiencies were detected; absolute values were well above the 30% cutoff considered adequate for normal haemostasis. No α2AP deficiencies were detected. These data suggest that in BDUC patients, measuring FXIII or AP activity is of limited value.</abstract><cop>England</cop><pub>Wiley Subscription Services, Inc</pub><pmid>38812123</pmid><doi>10.1111/hae.15059</doi><tpages>5</tpages><orcidid>https://orcid.org/0000-0002-2762-6033</orcidid><orcidid>https://orcid.org/0000-0003-3251-8595</orcidid><orcidid>https://orcid.org/0009-0003-9675-122X</orcidid><orcidid>https://orcid.org/0000-0002-6244-0429</orcidid><orcidid>https://orcid.org/0000-0001-7126-6613</orcidid><orcidid>https://orcid.org/0000-0003-3242-5524</orcidid><oa>free_for_read</oa></addata></record> |
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| ispartof | Haemophilia : the official journal of the World Federation of Hemophilia, 2024-07, Vol.30 (4), p.998-1002 |
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| source | Wiley Online Library Journals Frontfile Complete |
| subjects | alpha‐2‐antiplasmin Bleeding bleeding disorder of unknown cause (BDUC) Blood coagulation Coagulation factor XIII Coagulation factors factor XIII deficiency haemorrhagic disorders Hematological diseases primary haemostasis |
| title | Limited value of testing for factor XIII and α2‐antiplasmin deficiency in patients with a bleeding disorder of unknown cause |
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