A case of thrombotic thrombocytopenic purpura associated with COVID-19

Nicolotti, Davide; Bignami, Elena; Rossi, Sandra; Vezzani, Antonella

doi:10.1007/s11239-020-02362-7

Cited by 18 publications

(27 citation statements)

References 4 publications

(3 reference statements)

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“…Conversely to all the published cases, our patient presented with normal hemoglobin, however his severe thrombocytopenia is what pulled the trigger to evaluate his peripheral blood smear, TTP with normal Hb on presentation is rare, however it was reported in the literature [15] . The response to classic acute TTP treatment (Plasma exchange, Steroids and Rituximab) in our patient and the reported cases is reassuring, however with severe COVID-19 infection, specifically with respiratory failure the treatment might be prolonged, for example, Nicolotti, D., et al reported 14 plasma exchange over 18 days were required until the patient completely recovered [14] .…”

Section: Discussionsupporting

confidence: 69%

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COVID-19 infection triggering Thrombotic Thrombocytopenic Purpura

Alhomoud

Alhobayb

Armitage

2021

IDCases

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We present a 62-year-old gentleman with history of Crohn’s disease, G6PD deficiency, who presented with immune-mediated thrombotic thrombocytopenia purpura (iTTP) one week after the diagnosis of COVID-19 infection. He was admitted with worsening dyspnea, acute renal failure, and profound thrombocytopenia with marked schistocytosis on peripheral smear. ADAMTS13 level was severely deficient. He was treated with oral prednisone, plasma exchange and rituximab with complete clinical resolution. Given the temporal association of this recurrent episode of iTTP with COVID-19 infection and no other discernible cause, COVID-19 infection was the most likely trigger.

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Section: Discussionsupporting

confidence: 69%

“…There is increasing evidence that it is also associated with TTP. We utilized PubMed and Google Scholar to conduct a search and identified six cases of TTP following COVID-19 infection [9] , [10] , [11] , [12] , [13] , [14] . We summarized those cases in Table 1 .…”

Section: Discussionmentioning

confidence: 99%

COVID-19 infection triggering Thrombotic Thrombocytopenic Purpura

Alhomoud

Alhobayb

Armitage

2021

IDCases

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“…TTP bouts associated with COVID-19 have been reported earlier in several case reports. [5][6][7] In contrast to these reports, our patient did not take additional concomitant medication, for example, hydroxychloroquine for the treatment of COVID-19 that might have contributed to the onset or continuance of the iTTP relapse. The patient was treated effectively only with a monotherapy with caplacizumab on the first day of admission and a dual therapy of caplacizumab and corticosteroids during further hospitalization.…”

Section: Discussionmentioning

confidence: 96%

COVID-19 as a Potential Trigger for Immune Thrombotic Thrombocytopenic Purpura and Reason for an Unusual Treatment: A Case Report

et al. 2021

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Immune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, inhibitors, substituting ADAMTS13, and the accompaniment of an immunosuppressive treatment with steroids. Recently, caplacizumab was approved for iTTP. Caplacizumab is a nanobody binding the A1 domain of VWF, blocking its interaction with glycoprotein Ib–IX–V platelet receptor and therefore preventing platelet aggregation. VWF activities may serve as therapeutic drug monitoring of caplacizumab, whereas ADAMTS13 activities may be used for biomarkers to guide caplacizumab treatment modalities and overall treatment duration. Additional immunosuppressive treatment by inhibiting autoantibody formation (e.g., the use of Rituximab, a chimeric monoclonal antibody directed against the B-cell antigen CD20) is a further treatment option. Infections are well-known causes for an acute episode for patients with iTTP. The novel SARS-CoV-2 virus is mainly associated with acute respiratory distress as well as diffuse endothelial inflammation and increased coagulopathy. However, little is known about an infection with SARS-CoV-2 virus triggering iTTP relapses. We herein report the case of an acute iTTP episode accompanying a SARS-CoV-2 infection.

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“…ADAMTS13 (A disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13) is a protease that cleaves the large multimers of von Willebrand factor (vWF) which mediate platelet adhesion and activation. ADAMTS13 activity regulates the size of vWF multimers by cleaving the A2 domain of vWF to prevent platelet driven thrombus formation in physiological conditions [1].…”

Section: Introductionmentioning

confidence: 99%

“…Studies are ongoing to understand the physiology of microvascular thrombus formation in COVID-19 and one of the possible mechanisms is imbalance in the vWF/ADAMTS13 levels [1,[7][8][9][10]. A few reports also suggest the presence of TTP in COVID-19 patients [11][12][13][14]. We conducted a pilot study to establish the ADAMTS13 factor activity and inhibitor levels in patients with severe SARS CoV-2 infection and to generate evidence for further studies.…”

Section: Introductionmentioning

confidence: 99%

ADAMTS13 factor deficiency in severe COVID-19 may not be immune mediated – report from a pilot study

George

Chenna

Mohan

et al. 2022

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BACKGROUND: The assessment of ADAMTS13 factor activity and inhibitor levels was conducted in severe COVID-19 patients as an observational study. RESULTS: A total of 14 patients were included and the average ADAMTS13 activity level at the time of admission was 28.54±30.74% (range 1.83–86.67%) which was reduced compared to controls (88.09±14.77). Nine patients had reduced ADAMTS13 factor activity (<40%) and 77.7% among them had severe deficiency (<10% activity). ADAMTS13 inhibitor was positive (>15 IU/mL) only in two patients and an overall mean value was 8.15±5.8. Elevated D-Dimer and length of hospital stay had significant correlation with ADAMTS13 activity (–0.247 and 0.306 respectively). No features of thrombotic microangiopathy were observed and hence no plasma exchange was performed. CONCLUSION: Reduced ADAMTS13 factor activity without inhibitor development may give a clue to the disease progress in COVID19.

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A case of thrombotic thrombocytopenic purpura associated with COVID-19

Cited by 18 publications

References 4 publications

COVID-19 infection triggering Thrombotic Thrombocytopenic Purpura

COVID-19 infection triggering Thrombotic Thrombocytopenic Purpura

COVID-19 as a Potential Trigger for Immune Thrombotic Thrombocytopenic Purpura and Reason for an Unusual Treatment: A Case Report

ADAMTS13 factor deficiency in severe COVID-19 may not be immune mediated – report from a pilot study

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