A Case of COVID-19 Induced Thrombotic Thrombocytopenic Purpura

Abstract

Thrombotic Thrombocytopenic Purpura (TTP) is a challenging thrombotic diathesis which requires prompt diagnosis and therapeutic intervention in order to avoid life-threatening consequences. There are two forms of TTP, congenital and acquired, with the acquired form constituting about 90% of cases. Both forms are associated with a deficiency of ADAMTS-13, a metalloproteinase enzyme responsible for cleaving ultra-large von Willebrand factor (uLvWF), preventing its pathologic accumulation. Within the last year, many of the diverse and serious effects of the COVID-19 virus have come to recognition, with some of the most dire consequences involving devastating vascular and hematologic complications. As with many viruses, it seems that the endothelium and the vasculature are often prime targets.Here, we report a case of a 30 year old male who was diagnosed with TTP approximately one week after a positive COVID-19 test result. He responded appropriately to plasma exchange (PLEX), caplacizumab, and steroids.We believe it is important to investigate a potential link between these two conditions, as TTP has significant morbidity and mortality risk if left unattended. We hope that our report will contribute to a better understanding of this potential link.

COVID‐19 vaccine (mRNA BNT162b2) and COVID‐19 infection‐induced thrombotic thrombocytopenic purpura in adolescents

“… 10 A similar disease time course has been described in adults after COVID‐19 and influenza infections. 11 , 12 In the case of influenza‐induced TTP, the authors propose that the infection triggers the anti‐ADAMTS13 IgG inhibitor 12 ; development of inhibitory antibodies after COVID‐19 vaccination may be particularly robust in adolescents with predisposition to autoimmunity.…”

COVID-19 Associated Thrombotic Thrombocytopenic Purpura: A Case Report and Systematic Review

“…Commentaries, review articles and abstracts without full texts were excluded as well. We included 8 articles including the data of 11 patients with COVID-19 associated TTP [5][6][7][8][9][10][11][12] as depicted in PRISMA flow diagram (Figure 1).…”

“…All of the included eleven cases had a reported ADAMTS13 activity of less than 10%. Eight (72%) cases had elevated ADAMTS 13 inhibitor or antibody levels with the exception of three cases [6,11,12]. The pertinent laboratory investigations with other inflammatory markers and radiological findings are as summarized in Table 2…”

“…Two cases did not mention the steroid dosage used. Out of 11 cases, six received RTX (54.5%) [5,7,9,10,12] while three received CZB (27.3%), including two cases that received it in addition to RTX [9,10] (Table 2). Almost half of the patients did not receive additional immunosuppressants with PLEX despite more than 5 sessions of PLEX.…”

“…Majority of cases (90.9%) had favorable outcome with no mention of early relapse. There was one reported death [12]. The median length of hospital stay was 18.5 days (…”