ADAMTS13, VWF, and Endotoxin Are Interrelated and Associated with the Severity of Liver Cirrhosis via Hypercoagulability

Takaya, Hiroaki; Namisaki, Tadashi; Asada, Shohei; Iwai, Satoshi; Kubo, Takahiro; Suzuki, Junya; Enomoto, Masahide; Tsuji, Yosuke; Fujinaga, Yukihisa; Nishimura, Norihisa; Sawada, Yasuhiko; Kaji, Kosuke; Kawaratani, Hideto; Moriya, Kyoji; Akahane, Takemi; Matsumoto, Masanori; Yoshiji, Hitoshi

doi:10.3390/jcm11071835

Cited by 12 publications

(9 citation statements)

References 51 publications

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“…ADAMTS-13:AC levels are inversely proportional to the severity of LC, leading to the observed imbalance between the decreased ADAMTS-13:AC level and the increased vWF:Ag level in patients with LC. ADAMTS-13 and vWF are associated with the severity of LC via hypercoagulability, which represents a significant risk factor for PVT development [ 26 ]. Regarding the characteristic background, the reason for the differences in INR, PT, and D-dimer levels between studies remains unclear.…”

Section: Discussionmentioning

confidence: 99%

ADAMTS-13: A Prognostic Biomarker for Portal Vein Thrombosis in Japanese Patients with Liver Cirrhosis

Suzuki,

Namisaki,

Takya

et al. 2024

IJMS

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Portal vein thrombosis (PVT), one of the most prevalent hepatic vascular conditions in patients with liver cirrhosis (LC), is associated with high mortality rates. An imbalance between a disintegrin-like metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS-13) enzyme and von Willebrand factor (VWF) is responsible for hypercoagulability, including spontaneous thrombus formation in blood vessels. Herein, we aimed to identify potential prognostic and diagnostic biomarkers in Japanese patients with LC and PVT. In total, 345 patients were divided into two groups: 40 patients who developed PVT (PVT group) and 305 who did not develop PVT (NPVT group). Among the 345 patients with LC, 81% (279/345) were deemed ineligible due to the presence of preventive comorbidities, active or recent malignancies, and organ dysfunction. The remaining 66 patients were divided into two groups: the PVT group (n = 33) and the NPVT group (n = 33). Plasma ADAMTS-13 activity (ADAMTS-13:AC) and the vWF antigen (VWF:Ag) were measured using enzyme-linked immunosorbent assays. Contrast-enhanced, three-dimensional helical computed tomography (CT) was used to detect and characterize PVT. ADAMTS-13:AC was significantly lower in the PVT group than in the NPVT group. No significant differences in plasma vWF:Ag or liver stiffness were observed between the two groups. ADAMTS-13:AC of <18.8 was an independent risk factor for PVT on multivariate analyses (odds ratio: 1.67, 95% confidence interval: 1.21–3.00, p < 0.002). The receiver operating characteristic analysis of ADAMTS-13:AC revealed an area under the curve of 0.913 in PVT detection. Patients with PVT having ADAMTS-13:AC ≥18.8 (n = 17) had higher albumin levels and better prognoses than those with ADAMTS-13:AC <18.8 (n = 16). No significant correlations of ADAMTS-13:AC levels with either fibrin degradation product or D-dimer levels were observed. ADAMTS-13:AC levels could be potential diagnostic and prognostic biomarkers for PVT in Japanese patients with LC.

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Section: Discussionmentioning

confidence: 99%

ADAMTS-13: A Prognostic Biomarker for Portal Vein Thrombosis in Japanese Patients with Liver Cirrhosis

Suzuki,

Namisaki,

Takya

et al. 2024

IJMS

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“…A subclinical TTP-like state without disseminated intravascular coagulation (DIC) has also been described in these patients. However, very low ADAMTS13 activity, as seen in TTP, is exceedingly rare even among patients with essentially absent hepatic function [9][10][11][12]. As such, patients with findings of severe ADAMTS13 deficiency in the setting of microangiopathic hemolytic anemia should be treated for iTTP, even in the setting of Child-Pugh class C cirrhosis, considering the high mortality risk of leaving iTTP untreated.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura in the Setting of Cirrhosis and Baseline Thrombocytopenia

Venkatesh,

Bennett,

Halkidis

2024

Cureus

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The management of immune thrombotic thrombocytopenic purpura (iTTP) has evolved significantly over the past several years. However, despite recent advances, there are limited tools available for patients with comorbidities that preclude either the utilization of available treatment modalities or evidence-based laboratory target levels. Literature to guide the management of such patients is sparse at best, and many complications associated with pre-existing comorbidities in the context of iTTP have not been reported. Here we describe the case of a patient with severe thrombocytopenia at baseline due to liver cirrhosis who developed iTTP. The challenges of the case in terms of pursuing disease-directed treatment, defining laboratory parameters to guide treatment, and mitigating the risks of bleeding and disease exacerbation are discussed. We offer our perspective in treating iTTP in the setting of severe baseline thrombocytopenia and high bleeding risk.

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“…A delay in the sample collection after initiating TPE can also affect the results. Moreover, low ADAMTS13 levels are seen in cases with liver coagulopathy, disseminated intravascular coagulopathy (DIC), and sepsis 28‐30 . Suggested improvements can be made in allowing the test to be done after regular laboratory working hours and agreements for feasible sample shipments to a specialized laboratory.…”

Section: Discussionmentioning

confidence: 99%

A Saudi multicenter experience on therapeutic plasma exchange for patients with thrombotic thrombocytopenic purpura: A call for national registry

Radhwi,

Badawi,

Almarzouki

et al. 2023

J of Clinical Apheresis

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BackgroundThe improvement in the clinical care for patients with thrombotic thrombocytopenic purpura (TTP) is evolving, and many efforts are being put to standardize it. Here, we aimed to assess the provided care at a national level and identify deficiencies.MethodsA national Saudi retrospective descriptive study was carried out at six tertiary referral centers and included all patients who underwent therapeutic plasma exchange (TPE) for the diagnosis of TTP between May 2005, and July 2022. Collected information included demographic data, clinical features on presentation, and the results of laboratory investigations at admission and discharge. In addition, the number of TPE sessions, days till the first session of TPE, usage of immunological agents, and clinical outcomes were all collected.ResultsOne hundred patients were enrolled, predominantly female (56%). The mean age was 36.8 years. At diagnosis, 53% of patients showed neurological involvement. The mean platelet count at presentation was 21 × 109/L. All patients had anemia (mean hematocrit 24.2%). Schistocytes were present in the peripheral blood film of all patients. The mean number of TPE rounds was 13 ± 9.3, and the mean days to start TPE since admission for the first episode was 2.5 days. ADAMTS13 level was measured in 48% of patients and was significantly low in 77% of them. Assessing for clinical TTP scores, 83%, 1000%, 64% of eligible patients had an intermediate/high PLASMIC, FRENCH, and Bentley scores, respectively. Caplacizumab was used on only one patient, and rituximab was administered to 37% of patients. A complete response for the first episode was achieved in 78% of patients. The overall mortality rate was 25%. Neither time to TPE, the use of rituximab or steroid affected survival.ConclusionsOur study shows an excellent response to TPE with a survival rate approximate to the reported international literature. We observed a deficiency in using validated scoring systems in addition to confirming the disease by ADAMTS13 testing. This emphasizes the need for a national registry to facilitate proper diagnosis and management of this rare disorder.

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ADAMTS13, VWF, and Endotoxin Are Interrelated and Associated with the Severity of Liver Cirrhosis via Hypercoagulability

Cited by 12 publications

References 51 publications

ADAMTS-13: A Prognostic Biomarker for Portal Vein Thrombosis in Japanese Patients with Liver Cirrhosis

ADAMTS-13: A Prognostic Biomarker for Portal Vein Thrombosis in Japanese Patients with Liver Cirrhosis

Thrombotic Thrombocytopenic Purpura in the Setting of Cirrhosis and Baseline Thrombocytopenia

A Saudi multicenter experience on therapeutic plasma exchange for patients with thrombotic thrombocytopenic purpura: A call for national registry

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