COVID-19 Presented with Deep Vein Thrombosis in a Patient with Paroxysmal Nocturnal Haemoglobinuria

Abstract

Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired clonal haematological disease characterized by complement-mediated haemolysis, bone marrow failure and venous thrombosis. Anticomplement therapy eculizumab improves survival and reduces complications. Severe acute respiratory distress syndrome corona virus 2 (SARS-CoV-2) disease 2019 (COVID-19) is associated with high incidence of both venous and arterial thrombosis in hospitalized patients with pneumonia. Deep venous thrombosis (DVT) as the presenting symptom of COVID-19 is a rare event. We describe a well-controlled PNH patient on eculizumab for more than 5 years who presented with DVT, while on warfarin, as the first sign of COVID-19. To our knowledge, this is the first described case of DVT in a PNH patient with COVID-19.

Severe hemolytic exacerbations of Chinese PNH patients infected SARS‐CoV‐2 Omicron

“…The literature 10,[12][13][14]16 documented less frequent hemolysis following SARS-CoV-2 infection in PNH patients who regularly used complement inhibitors. Compared with cases with LDH values reported in the literature, hemolysis in current study was more severe (LDH 7.47 × ULN vs. 2.04 × ULN, p < .001).…”

“…In this study, there was no thromboembolism, but d ‐dimer values of a half of patients were beyond the ULN. Zlatko et al 16 reported 1 PNH patient who presented with deep vein thrombosis as the first sign of COVID‐19. SARS‐CoV‐2 may increase thrombophilia in PNH in the context of multiple triggers, such as increased inflammatory factors, endothelial injury, platelet, and thrombin activations.…”

“…Specific antibody directed against the receptor‐binding domain of the spike protein initiates the classical pathway, the binding of mannose‐binding lectin with SARS‐CoV‐2 spike protein triggers the lectin pathway and SARS‐CoV‐2 spike protein may dysregulate the alternative pathway by binding heparan sulfate and competing with factor H, which is a negative regulator of complement activity. 5 , 6 After SARS‐CoV‐2 infection, patients with PNH more likely suffered the hemolysis, 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 patients usually presented visible hemoglobinuria and a small number patients showed pancytopenia. 17 Terminal complement inhibitors (such as eculizumab) are promising in COVID‐19 treatment by blocking the formation of membrane attack complex and reducing proinflammatory and prothrombotic influence.…”

Pancytopenia in a Case of Aplastic Anaemia/Paroxysmal Nocturnal Haemoglobinuria Unmasked by SARS-CoV-2 Infection: A Case Report

“…Interestingly, a correlation between SARS-CoV-2 infection and PNH appears to be emerging. In fact, from the beginning of the pandemic, many cases of clinical deterioration and increased haemolysis in patients with a known diagnosis of PNH following infection with SARS-CoV-2 have been reported [11][12][13][14][15][16][17][18][19][20][21]. There is one case report of a COVID-19 infection which presented with deep vein thrombosis in a patient with PNH [20].…”

“…In fact, from the beginning of the pandemic, many cases of clinical deterioration and increased haemolysis in patients with a known diagnosis of PNH following infection with SARS-CoV-2 have been reported [11][12][13][14][15][16][17][18][19][20][21]. There is one case report of a COVID-19 infection which presented with deep vein thrombosis in a patient with PNH [20]. Hines et al have described a case in which a patient received a diagnosis of PNH during SARS-CoV-2 infection, without typical symptoms and in which, considering the temporal correlation, it appears possible that there was a direct role of the virus in the pathogenesis of the disease [21].…”