ADAMTS13 activity levels in patients with human immunodeficiency virus‐associated thrombotic microangiopathy and profound CD4 deficiency

Park, Yara A.; Hay, Shauna N.; Brecher, Mark E.

doi:10.1002/jca.20189

Cited by 17 publications

(20 citation statements)

References 31 publications

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“…The association of HIV and TMA is rare but well known; however, HIV-associated TMA is postulated to have a different pathophysiology than idiopathic TTP. 13 Response to infection may generate antibodies that cross-react with platelet antigens. Platelet production may be impaired by infection of megakaryocyte bone marrowϪdependent progenitor cells and decreased production of thrombopoietin.…”

Section: Discussionmentioning

confidence: 99%

Suspicious Neuroimaging Pattern of Thrombotic Microangiopathy

Ellchuk¹,

Shah²,

Hewlett³

et al. 2011

AJNR Am J Neuroradiol

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SUMMARY:The TMAs are a group of microvascular occlusive disorders characterized by thrombocytopenia and intravascular hemolysis. Literature review reveals a spectrum of neuroimaging findings, including a single case report of multifocal hemorrhagic infarctions. We present a series of 12 patients with TMA demonstrating a similar pattern of multifocal cortical and subcortical hemorrhagic infarctions.ABBREVIATIONS: ADC ϭ apparent diffusion coefficient; AML ϭ acute myelogenous leukemia; CLL ϭ chronic lymphocytic leukemia; CNS ϭ central nervous system; CTV ϭ CT venography; DIC ϭ disseminated intravascular coagulation; DWI ϭ diffusion-weighted imaging; GRE ϭ gradient recalled-echo; HIV ϭ human immunodeficiency virus; HUS ϭ hemolytic uremic syndrome; mHTN ϭ malignant hypertension; MRI ϭ MR imaging; MRV ϭ MR venography; PRES ϭ posterior reversible encephalopathy syndrome; SWI ϭ susceptibility-weighted imaging; TMA ϭ thrombotic microangiopathy; TTP ϭ thrombotic thrombocytopenic purpura; vWF ϭ von Willebrand factor T MA was defined by Symmers in 1952 1 as a lesion of arteriole and capillary wall thickening with swelling or detachment of endothelial cells from the basement membrane, accumulation of material in the subendothelial space, and lodging of fibrin-platelet thrombi at the arteriocapillary junction.2 The mechanical injury to erythrocytes results in intravascular hemolysis and schistocytosis. The intraluminal platelet thrombosis results in partial or complete obstruction of the vessel lumina.

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Section: Discussionmentioning

confidence: 99%

Suspicious Neuroimaging Pattern of Thrombotic Microangiopathy

Ellchuk¹,

Shah²,

Hewlett³

et al. 2011

AJNR Am J Neuroradiol

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“…A recent summary presented at the 2012 American Society for Apheresis (ASFA) Consensus Conference on Classification, Diagnosis, Management, and Future Research in TTP showed that the autoantibody is present in idiopathic disease in 51 to 93% of cases [30]. Not all adults with HIV-TTP reported in the literature however, show a significant decrease in the activity of ADAMTS13 [4,9,31,32]. The number of cases varies depending on the definition of HIV-TTP used in each study.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, we cannot be sure that all patients with HIV-HUS or other causes of MAHA were excluded from these studies. Alternatively, HIV-1 can play a unique role in the pathogenesis of HIV-TTP by causing widespread endothelial damage from direct viral or cytokine-mediated cell injury [4,9,32]. These changes cause the release of vWF triggering a localized coagulation cascade mainly in the renal and brain microvasculature, [4,31,32].…”

Section: Discussionmentioning

confidence: 99%

“…The rise in the platelet count in our patient was associated with a significant drop in HIV-1 RNA VL attributable to the re-initiation of ART and possibly, to the physical removal of viruses and circulating cytokines through the plasma exchange therapy. It is tempting to speculate that the removal of inflammatory cytokines and HIV virions through plasma exchange, could have reduced the endothelial damage and secondary release of vWF release [4,9,32]. Nonetheless, the more sustainable increase in platelet count was observed following the initiation of ART.…”

Section: Discussionmentioning

confidence: 99%

“…HIV-1 can trigger acute and lethal atypical episodes of the Hemolytic Uremic Syndrome (HUS) [2,3], and Thrombotic Thrombocytopenic Purpura (TTP) [4–8]. The main histological feature of HIV-associated HUS or TTP is microvascular thrombosis with endothelial damage, affecting predominately the kidney and brain, and potentially other tissues as well [2–9]. …”

Section: Introductionmentioning

confidence: 99%

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Hemorrhagic Stroke in an Adolescent Female with HIV-Associated Thrombotic Thrombocytopenic Purpura

et al. 2014

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HIV-1 infection can trigger acute episodes of Idiopathic Thrombocytoponic Purpura (ITP), and Thrombotic Thrombocytopenic Purpura (TTP), particularly in populations with advanced disease and poor adherence to antiretroviral therapy (ART). These diseases should be distinguished because they respond to different treatments. Previous studies done in adults with HIV-TTP have recommended the prompt initiation or re-initiation of ART in parallel with plasma exchange therapy to improve the clinical outcome of these patients. Here, we describe a case of HIV-TTP resulting in an acute hemorrhagic stroke in a 16 year old female with perinatally acquired HIV infection and non-adherence to ART, who presented with severe thrombocytopenia, microangiopathic hemolytic anemia, and a past medical history of HIV-ITP. Both differential diagnosis and treatments for HIV-ITP and HIV-TTP were considered simultaneously. A decrease in plasma ADAMTS13 activity (<5%) without detectable inhibitory antibodies confirmed the diagnosis of HIV-TTP. Re-initiation of ART and plasma exchange resulted in a marked decrease in the HIV-RNA viral load, recovery of the platelet count, and complete recovery was achieved with sustained virologic suppression.

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Is it HIV TTP or HIV‐associated thrombotic microangiopathy?

Brecher

Hay

Park

2008

J of Clinical Apheresis

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Thrombocytopenia is a common complication of Human Immunodeficiency Virus (HIV) infection. With advanced HIV disease, the presence of both thrombocytopenia and schistocytosis are frequently observed. In such cases, the diagnosis of HIV associated TTP is often considered. This article reviews emerging concepts of HIV associated microangiopathies. It concludes that the pathophysiology, in many cases seems to be distinct from idiopathic TTP (particularly with advanced HIV disease-<100 CD4/microliter). A sine que non for successful therapy of HIV-TMA appears to be the treatment of the underlying HIV infection.

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ADAMTS13 activity levels in patients with human immunodeficiency virus‐associated thrombotic microangiopathy and profound CD4 deficiency

Abstract: HIV-associated TMA is postulated to have a different pathophysiology than idiopathic TTP. This study supports that assumption because both patients exhibited many of the classic findings of TTP but did not have a deficiency of ADAMTS13.

Cited by 17 publications

References 31 publications

Suspicious Neuroimaging Pattern of Thrombotic Microangiopathy

Suspicious Neuroimaging Pattern of Thrombotic Microangiopathy

Hemorrhagic Stroke in an Adolescent Female with HIV-Associated Thrombotic Thrombocytopenic Purpura

Is it HIV TTP or HIV‐associated thrombotic microangiopathy?

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