ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE

Acquired FXIII deficiency has been described in association with malignancies or autoimmune disorders. We report two cases of acquired FXIII deficiency associated with hematologic malignancies. The first patient is a 60-year-old male with CMML who presented 4 weeks after confirming his diagnosis wit...

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Autores principales:	Alfadil Haroon, Ali Alahmari, Nadiah Alobaidi, Ahmed Syed Osman, Hazzaa Alzahrani
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/d9d562f0173246d1a888b085e6744707
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spelling	oai:doaj.org-article:d9d562f0173246d1a888b085e67447072021-11-10T04:35:43ZACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE2531-137910.1016/j.htct.2021.10.1031https://doaj.org/article/d9d562f0173246d1a888b085e67447072021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2531137921011780https://doaj.org/toc/2531-1379Acquired FXIII deficiency has been described in association with malignancies or autoimmune disorders. We report two cases of acquired FXIII deficiency associated with hematologic malignancies. The first patient is a 60-year-old male with CMML who presented 4 weeks after confirming his diagnosis with non-traumatic anterior abdominal wall hematoma. Workup revealed FXIII deficiency. He was treated with FXIII replacement and other supportive measures. The hematoma resolved and patient was maintained on factor replacement. Unfortunately, his disease transformed to AML and he succumbed to death after starting AML therapy despite achieving complete remission. The second patient is a 24-year-old male patient post haploidentical transplant for intermediate risk AML. He developed hemorrhagic cystitis day 36 post-transplant and non-traumatic subdural hematoma on day 60 post-transplant. Workup revealed FXIII deficiency. He was treated with factor replacement and the subdural hematoma resolved with improvement of the hemorrhagic cystitis. Both patients had RUNX1 mutation which regulates expression of F13A1 in megakaryocyte this can decreased platelet expression of F13A1 in patient with RUNX1 haplodeficiency which lead to platelet dysfunction. FXIII deficiency should be considered for patient with unexplained bleeding with normal routine workup.Alfadil HaroonAli AlahmariNadiah AlobaidiAhmed Syed OsmanHazzaa AlzahraniElsevierarticleDiseases of the blood and blood-forming organsRC633-647.5ENHematology, Transfusion and Cell Therapy, Vol 43, Iss , Pp S40-S41 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	Diseases of the blood and blood-forming organs RC633-647.5 Alfadil Haroon Ali Alahmari Nadiah Alobaidi Ahmed Syed Osman Hazzaa Alzahrani ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE
description	Acquired FXIII deficiency has been described in association with malignancies or autoimmune disorders. We report two cases of acquired FXIII deficiency associated with hematologic malignancies. The first patient is a 60-year-old male with CMML who presented 4 weeks after confirming his diagnosis with non-traumatic anterior abdominal wall hematoma. Workup revealed FXIII deficiency. He was treated with FXIII replacement and other supportive measures. The hematoma resolved and patient was maintained on factor replacement. Unfortunately, his disease transformed to AML and he succumbed to death after starting AML therapy despite achieving complete remission. The second patient is a 24-year-old male patient post haploidentical transplant for intermediate risk AML. He developed hemorrhagic cystitis day 36 post-transplant and non-traumatic subdural hematoma on day 60 post-transplant. Workup revealed FXIII deficiency. He was treated with factor replacement and the subdural hematoma resolved with improvement of the hemorrhagic cystitis. Both patients had RUNX1 mutation which regulates expression of F13A1 in megakaryocyte this can decreased platelet expression of F13A1 in patient with RUNX1 haplodeficiency which lead to platelet dysfunction. FXIII deficiency should be considered for patient with unexplained bleeding with normal routine workup.
format	article
author	Alfadil Haroon Ali Alahmari Nadiah Alobaidi Ahmed Syed Osman Hazzaa Alzahrani
author_facet	Alfadil Haroon Ali Alahmari Nadiah Alobaidi Ahmed Syed Osman Hazzaa Alzahrani
author_sort	Alfadil Haroon
title	ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE
title_short	ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE
title_full	ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE
title_fullStr	ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE
title_full_unstemmed	ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE
title_sort	acquired factor xiii deficiency with runx1 mutation, a report of two cases treated with factor xiii concentrate
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/d9d562f0173246d1a888b085e6744707
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ACQUIRED FACTOR XIII DEFICIENCY WITH RUNX1 MUTATION, A REPORT OF TWO CASES TREATED WITH FACTOR XIII CONCENTRATE

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