Does this patient with thrombotic thrombocytopenic purpura have a cardiac involvement?

Zafrani, Lara; Russell, Lene; Azoulay, Élie

doi:10.1007/s00134-018-5243-4

Cited by 3 publications

(2 citation statements)

References 20 publications

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“…In a study of 17 autopsied patients with TTP, 13 had evidence of extensive small‐vessel thromboses within the heart arterioles [ 30 ]. Case reports also describe cardiomyopathy associated with TTP in CAPS, with two of three specifically showing improvement with treatment of the underlying disease, including plasmapheresis [ 31 , 32 , 33 ]. Likewise, in Shiga toxin‐producing Escherichia coli ‐associated HUS, there have been described cases of dilated cardiomyopathy occurring in children and adults, with favorable cardiac outcomes after treatment [ 34 , 35 ].…”

Section: Discussionmentioning

confidence: 99%

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Hermel

Azam

et al. 2020

eJHaem

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Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare form of antiphospholipid syndrome, an autoimmune condition characterized by vascular thromboses, pregnancy loss, and antiphospholipid (aPL) antibodies. Diagnosis of CAPS relies on thrombosis of at least three different organs systems over 1 week, histopathological evidence of small vessel occlusion, and high aPL antibody titers. In a subset of precipitating circumstances, activation or disruption of endothelial cells in the microvasculature may occur along with cardiomyopathy. We present two cases of CAPS‐associated dilated cardiomyopathy at our institution, focusing on disease management, pathophysiology, and treatment. These patients were of Southeastern Asian descent, raising the possibility of genetic polymorphisms contributing to the development of cardiomyopathy. Both met CAPS criteria and both demonstrated clinicopathologic thrombotic microangiopathy (TMA) and complement activation and developed severe dilated cardiomyopathy with shock. Complement activation plays an important role in the development of CAPS and may be important in the pathogenesis of CAPS‐associated cardiomyopathy. Clinical suspicion for TMA as a pathophysiologic mechanism of unexplained heart failure in CAPS is important and increased awareness of cardiac side effects is necessary so that early treatment can be initiated to halt further cardiac and systemic complications.

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Section: Discussionmentioning

confidence: 99%

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Hermel

Azam

et al. 2020

eJHaem

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“…In general, the clinical evaluation of TTP patients at the time of hospital admission, and regularly thereafter, emphasizes cardiac and neurological assessment. Specifically, cardiac troponin levels are measured at diagnosis, followed by serial troponin levels, electrocardiography, and echocardiography as clinically indicated 22 . Increased cardiac troponin (>0.25 μg/L) appears to be associated with increased cardiac and cerebral involvement, ischemic stroke, and mortality in TTP, 5 although most patients with an increased level of cardiac troponin remain asymptomatic 5,10,23,24 …”

Section: Supportive Carementioning

confidence: 99%

Good practice statements (GPS) for the clinical care of patients with thrombotic thrombocytopenic purpura

Zheng

Vesely

Cataland

et al. 2020

Journal of Thrombosis and Haemostasis

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Background: Despite advances in treatment options for thrombotic thrombocytopenic purpura (TTP), there are still limited high quality data to inform clinicians regarding its management. Methods: In June 2018, the ISTH formed a multidisciplinary guideline panel to issue recommendations about treatment of TTP. The panel discussed 12 treatment questions related to both immune-mediated TTP (iTTP) and hereditary/congenital TTP (cTTP). The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including evidence-to-decision frameworks, to appraise evidence and formulate recommendations. Results: The panel agreed on eleven recommendations based on evidence ranging from very low to moderate certainty. For first episode and relapses of acute iTTP, the panel made a strong recommendation for the addition of corticosteroids to therapeutic plasma exchange (TPE), and a conditional recommendation for addition of rituximab and caplacizumab. For asymptomatic iTTP with low ADAMTS13, the panel made a conditional recommendation for rituximab outside of pregnancy, and for prophylactic TPE during pregnancy. For asymptomatic cTTP, the panel made a strong recommendation for prophylactic plasma infusion during pregnancy, but a conditional recommendation for plasma infusion or a wait and watch approach outside of pregnancy. Conclusions: The panel's recommendations are based on all the available evidence for the treatment effects of various approaches including suppressing inflammation, blocking platelet clumping, replacing the missing and/or inhibited ADAMTS13, and suppressing ADAMTS13 antibody production. There was insufficient evidence for further comparison of different treatment approaches, for which future high-quality

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Therapeutic plasma exchange in thrombotic thrombocytopenic purpura

Picod¹,

François

Coppo

2019

La Presse Médicale

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Does this patient with thrombotic thrombocytopenic purpura have a cardiac involvement?

Cited by 3 publications

References 20 publications

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review

Good practice statements (GPS) for the clinical care of patients with thrombotic thrombocytopenic purpura

Therapeutic plasma exchange in thrombotic thrombocytopenic purpura

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