Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases

Habe, Koji; Wada, Hideo; Ito-Habe, Naomi; Hatada, Tsuyoshi; Matsumoto, Toshifumi; Ohishi, Kohshi; Maruyama, Kazuo; Imai, Hiroshi; Mizutani, Hitoshi; Nobori, Tsutomu

doi:10.1016/j.thromres.2011.10.011

Cited by 63 publications

(75 citation statements)

References 22 publications

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“…These findings suggested that non-ADAMTS13 activity deficient TTP patients who suffer from an underlying disease are prone to suffer from a fibrinogen consumption commonly known as disseminated intravascular coagulation (DIC). Habe et al (18) recently reported that ADAMTS13/ VWF profiles may have important roles in the pathogenesis of DIC, and that the plasma levels of both ADAMTS13 and VWF propeptide are useful indicators for the diagnosis and prognosis of DIC. In contrast, the marker for immune thrombocytopenia (ITP), platelet-associated (PA) IgG, was present in significantly increased levels in all of our TTP patients, thereby indicating that PAIgG is not useful for differentiating between ITP and TTP.…”

Section: Discussionmentioning

confidence: 99%

Therapeutic Modality of 11 Patients with TTP in a Single Institution in Miyazaki from 2000 to 2011

et al. 2013

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Objective Thrombotic thrombocytopenic purpura (TTP) is a life-threatening generalized disease with pathological features that are termed thrombotic microangiopathies. Since the discovery of the von Willebrand factor-cleaving protease [a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)], it is widely known that approximately two-thirds of TTP patients have a severe deficiency of ADAMTS13 activity due to gene mutations or acquired autoantibodies to this enzyme. However, the remaining one-third of TTP patients have only moderately reduced or almost normal ADAMTS13 activity. To elucidate the clinical characteristics and outcomes of these two types of TTP, we have retrospectively analyzed the cases of acquired TTP patients treated in a single institution from 2000 to 2011. Methods Our case studies include 11 TTP patients, of which 5 were considered idiopathic and 6 had cases of TTP associated with underlying diseases such as non-Hodgkin lymphoma or connective tissue diseases. Results These patients were treated with a combination therapy of plasma exchange and steroids and with several adjunctive therapeutic regimens including the on-label use of cyclophosphamide and cyclosporine and the off-label use of high-dose steroid or immunoglobulin with rituximab. Splenectomies were not performed. As a result of these treatments, 6 out of the 7 patients with ADAMTS13 activity deficient TTP achieved a complete remission without relapse, but the remaining 4 patients with non-ADAMTS13 activity deficient TTP all died without complete remission. Conclusion We present herein the detailed clinical courses of 11 patients with TTP and address our experiences with the efficacy of various therapeutic regimens. This case-oriented study should be helpful to the physicians who directly care for TTP patients, and may provide a future direction for developing a more efficient treatment modality.

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Section: Discussionmentioning

confidence: 99%

Therapeutic Modality of 11 Patients with TTP in a Single Institution in Miyazaki from 2000 to 2011

et al. 2013

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“…39 Raised VWF levels and associated reduced ADAMTS 13 levels, are associated with worse outcomes in severe DIC. 40 For personal use only. on May 11, 2018.…”

Section: Comments About Patientmentioning

confidence: 99%

How I treat disseminated intravascular coagulation

Levi

Scully

2018

Blood

203

191

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Disseminated intravascular coagulation (DIC) is a condition characterised by systemic activation of coagulation, potentially leading to thrombotic obstruction of small and midsize vessels, thereby contributing to organ dysfunction. At the same time, ongoing consumption of platelets and coagulation proteins results in thrombocytopenia and low concentrations of clotting factors, which may cause profuse hemorrhagic complications. DIC is always secondary to an underlying condition, such as severe infections, solid or hematologic malignancies, trauma, or obstetric calamities. A reliable diagnosis of DIC can be made through simple scoring algorithms based on readily available routine hemostatic parameters. The cornerstone of supportive treatment of this coagulopathy is management of the underlying condition.

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“…Resuscitation with saline resulted in a ~4‐fold decrease in ADAMTS13 relative to baseline levels, whereas both Lo‐VitC‐treated and Hi‐VitC‐treated animals had relatively stable ADAMTS13 levels over the 6‐h study. Several studies in humans and animal models have reported decreases in ADAMTS13 levels and activity as a result of trauma, sepsis, and fluid resuscitation . Russel et al recently showed in 106 pediatric patients with severe trauma that ADAMTS13 levels were significantly lower upon admission and at 24 h compared to a control group of pediatric patients without trauma.…”

Section: Discussionmentioning

confidence: 99%

Temporal map of the pig polytrauma plasma proteome with fluid resuscitation and intravenous vitamin C treatment

Cudjoe

Hassan

Kang

et al. 2019

Journal of Thrombosis and Haemostasis

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Background Fluid resuscitation plays a prominent role in stabilizing trauma patients with hemorrhagic shock yet there remains uncertainty with regard to optimal administration time, volume, and fluid composition (e.g., whole blood, component, colloids) leading to complications such as trauma‐induced coagulopathies (TIC), acidosis, and poor oxygen transport. Synthetic fluids in combination with antioxidants (e.g., vitamin C) may resolve some of these problems. Objectives We applied quantitative mass spectrometry‐based proteomics [liquid chromatography‐mass spectrometry (LC‐MS/MS)] to map the effects of fluid resuscitation and intravenous vitamin C (VitC) in a pig model of polytrauma (hemorrhagic shock, tissue injury, liver reperfusion, hypothermia, and comminuted bone fracture). The goal was to determine the effects of VitC on plasma protein expression, with respect to changes associated with coagulation and trauma‐induced coagulopathy (TIC). Methods Longitudinal blood samples were drawn from nine male Sinclair pigs at baseline, 2 h post trauma, and 0.25, 2, and 4 h post fluid resuscitation with 500 mL hydroxyethyl starch. Pigs were treated intravenously (N = 3/treatment group) with saline, 50 mg VitC/kg (Lo‐VitC), or 200 mg VitC/kg (Hi‐VitC) during fluid resuscitation. Results A total of 436 plasma proteins were quantified of which 136 changed following trauma and resuscitation; 34 were associated with coagulation, complement cascade, and glycolysis. Unexpectedly, Lo‐VitC and Hi‐VitC treatments stabilized ADAMTS13 levels by ~4‐fold (P = .056) relative to saline and enhanced ADAMTS13/von Willebrand factor (VWF) cleavage efficiency based on LC‐MS/MS evidence for the semitryptic VWF cleavage product (VWF1275‐1286). Conclusions This study provides the first comprehensive map of trauma‐induced changes to the plasma proteome, especially with respect to proteins driving the development of TIC.

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Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases

Cited by 63 publications

References 22 publications

Therapeutic Modality of 11 Patients with TTP in a Single Institution in Miyazaki from 2000 to 2011

Therapeutic Modality of 11 Patients with TTP in a Single Institution in Miyazaki from 2000 to 2011

How I treat disseminated intravascular coagulation

Temporal map of the pig polytrauma plasma proteome with fluid resuscitation and intravenous vitamin C treatment

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