TY - JOUR
T1 - Low FXIII activity levels in patients with vascular anomalies, not in patients with trauma-induced coagulopathy
AU - Otsubo, Hiroaki
AU - Terashima, Tsuguaki
AU - Terashima, Mai
AU - Gao, Siqiang
AU - Ogawa, Mika
AU - Kato, Hidefumi
AU - Takeshita, Kyosuke
AU - Mizuno, Shohei
AU - Murakami, Satsuki
AU - Takakmi, Akiyoshi
AU - Matsuyama, Katsuhiko
AU - Furukawa, Hiroshi
AU - Watanabe, Eizo
AU - Nakayama, Takayuki
N1 - Publisher Copyright:
© 2026 Otsubo et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
PY - 2026/4
Y1 - 2026/4
N2 - Patients with localized intravascular coagulopathy (LIC) related to vascular anomalies occasionally suffer from hemorrhage and delayed wound healing, presumably due to consumptive reduction of fibrinogen and platelets. However, LIC patients with normal levels of platelets and fibrinogen can be hemorrhagic for unknown reasons. These facts led us to hypothesize that factor XIII (FXIII) is decreased since FXIII plays an important role in hemostasis, tissue repair, and wound healing. We collected plasma from 17 patients with LIC (9 with aneurysm, 8 with vessel malformation) and 7 patients with trauma-induced coagulopathy (TIC), which showed a fibrinolytic phenotype similar to LIC, and measured FXIII levels. FXIII levels were considerably decreased (7–53%, mean 29.4%) in LIC patients. 13 of 17 patients showed bleeding tendency even though DIC scores were low. However, FXIII levels were only slightly decreased (39–98%, mean 67.4%) in TIC patients. We also investigated the correlation between FXIII activity and coagulation parameters. FXIII activity most strongly correlated with fibrinogen content (R=0.882, P<0.001) in LIC patients. FXIII levels below 50% were observed when fibrinogen was less than 200 mg/dL. Based on these findings, we successfully treated two LIC patients with FXIII concentrates. Decreased FXIII activity was specific to LIC, not TIC. Testing for FXIII activity should be considered in LIC patients even with normal fibrinogen levels, who are scheduled to undergo any invasive procedure, since FXIII supplementation is occasionally required for them.
AB - Patients with localized intravascular coagulopathy (LIC) related to vascular anomalies occasionally suffer from hemorrhage and delayed wound healing, presumably due to consumptive reduction of fibrinogen and platelets. However, LIC patients with normal levels of platelets and fibrinogen can be hemorrhagic for unknown reasons. These facts led us to hypothesize that factor XIII (FXIII) is decreased since FXIII plays an important role in hemostasis, tissue repair, and wound healing. We collected plasma from 17 patients with LIC (9 with aneurysm, 8 with vessel malformation) and 7 patients with trauma-induced coagulopathy (TIC), which showed a fibrinolytic phenotype similar to LIC, and measured FXIII levels. FXIII levels were considerably decreased (7–53%, mean 29.4%) in LIC patients. 13 of 17 patients showed bleeding tendency even though DIC scores were low. However, FXIII levels were only slightly decreased (39–98%, mean 67.4%) in TIC patients. We also investigated the correlation between FXIII activity and coagulation parameters. FXIII activity most strongly correlated with fibrinogen content (R=0.882, P<0.001) in LIC patients. FXIII levels below 50% were observed when fibrinogen was less than 200 mg/dL. Based on these findings, we successfully treated two LIC patients with FXIII concentrates. Decreased FXIII activity was specific to LIC, not TIC. Testing for FXIII activity should be considered in LIC patients even with normal fibrinogen levels, who are scheduled to undergo any invasive procedure, since FXIII supplementation is occasionally required for them.
UR - https://www.scopus.com/pages/publications/105035712223
U2 - 10.1371/journal.pone.0346724
DO - 10.1371/journal.pone.0346724
M3 - Article
C2 - 41984884
AN - SCOPUS:105035712223
SN - 1932-6203
VL - 21
JO - PLoS ONE
JF - PLoS ONE
IS - 4 April
M1 - e0346724
ER -