COVID19 infection in a patient undergoing treatment for Paroxysmal Nocturnal Hemoglobinuria (PNH) with Ravulizumab

Shikdar, Sufana; Borogovac, Azra; Mohamad, Elabdallah; Khawandanah, Mohamad

doi:10.1186/s12959-021-00330-6

Cited by 6 publications

(9 citation statements)

References 16 publications

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“…However, recently published data indicate that patients with PNH may only present with mild symptoms of COVID-19 despite or even because of eculizumab treatment [ 78 – 81 ]. Further studies are needed to validate these observations [ 78 – 81 ].…”

Section: Resultsmentioning

confidence: 99%

“…Thromboprophylaxis in the context of C5 inhibition should be considered in patients with PNH and severe COVID-19 symptoms requiring hospitalization [78][79][80][81]. Note that patients with PNH and a history of thrombosis or other thrombophilic markers should receive secondary thromboprophylaxis while on C5 inhibition therapy, regardless of whether or not they have COVID-19.…”

Section: Other Considerationsmentioning

confidence: 99%

“…Eculizumab is currently used off-label to treat COVID-19, but its efficacy in this setting has yet to be proven [79]. However, recently published data indicate that patients with PNH may only present with mild symptoms of COVID-19 despite or even because of eculizumab treatment [78][79][80][81]. Further studies are needed to validate these observations [78][79][80][81].…”

Section: Other Considerationsmentioning

confidence: 99%

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Complement Inhibition in Paroxysmal Nocturnal Hemoglobinuria (PNH): A Systematic Review and Expert Opinion from Central Europe on Special Patient Populations

et al. 2023

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Introduction Hemolysis in paroxysmal nocturnal hemoglobinuria (PNH) is complement-mediated due to the lack of complement inhibitors in the hemopoietic cell membranes, making complement inhibition the best approach to manage PNH. Three complement inhibitors are approved by the European Medicines Agency as targeted therapy for PNH: eculizumab and ravulizumab, two humanized monoclonal antibodies targeting the same complement 5 (C5) epitope, approved in 2007 and 2019, respectively, and the more recently approved cyclic peptide, the complement 3 (C3) inhibitor pegcetacoplan. Although national and international PNH treatment guidelines exist, they do not take into consideration the latest clinical trial evidence. Given the lack of evidence-based data for some clinical situations encountered in real life, we identified specific populations of patients who may benefit from switching to proximal C3 from terminal C5 inhibition. Methods The expert recommendations presented here were created using a Delphi-like process by a group of expert PNH specialists across Central Europe. Based on an initial advisory board meeting discussion, recommendations were prepared and reviewed as part of a Delphi survey to test agreement. Results Using a systematic approach, literature databases were searched for relevant studies, and 50 articles were reviewed by the experts and included as supporting evidence. Conclusion Implementation of these recommendations uniformly across healthcare institutions will promote the best use of complement inhibition in managing PNH, and has the potential to positively impact patient outcomes in Central Europe and worldwide. Supplementary Information The online version contains supplementary material available at 10.1007/s12325-023-02510-4.

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Section: Resultsmentioning

confidence: 99%

Section: Other Considerationsmentioning

confidence: 99%

Section: Other Considerationsmentioning

confidence: 99%

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Complement Inhibition in Paroxysmal Nocturnal Hemoglobinuria (PNH): A Systematic Review and Expert Opinion from Central Europe on Special Patient Populations

et al. 2023

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“…Approximately, 35% of patients die within 5 years if untreated due to thrombosis and related complications. [10,11] The complement system activation is a critical component in the sequalae of COVID19 infection. Evidence suggests that severe outcomes in COVID19 infection are attributed to the excessive activation of the complement cascade leading to acute lung injury and associated is with an increased prothrombotic state.…”

Section: Discussionmentioning

confidence: 99%

COVID19 infection in a patient with paroxysmal nocturnal hemoglobinuria

Sokol¹,

Nehaj

Mokáň

et al. 2021

Medicine

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Introduction: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired, life-threatening hemopoietic stem cell disorder characterized by the triad of hemolytic anemia, thrombosis, and impaired bone marrow function. Evidence suggests that severe outcomes in COVID19 infection are attributed to the excessive activation of the complement cascade leading to acute lung injury and associated is with an increased prothrombotic state. Patient concerns: A 27-year-old Caucasian man with PNH presented to the Emergency Department of our hospital with acute onset shortness of breath, cough and blood in urine. Diagnosis: The patient was diagnosed with acute hemolytic exacerbation of PNH complicated with moderate COVID19 pneumonia. Outcomes: The patient was initiated with an anticoagulant unfractionated heparin, dexamethasone, and cefuroxime injection. His symptoms quickly resolved, and he was discharged after 5 days. Conclusion: The complement system activation is a critical component in the sequalae of COVID19 infection. Evidence suggests that severe outcomes in COVID19 infection are attributed to the excessive activation of the complement cascade leading to acute lung injury and associated is with an increased prothrombotic state. Notably, C5a concentration was noted to be higher in patients with COVID19 infection. The use of complement inhibitors to attenuate immune mediated damage in COVID19 nevertheless represents a very interesting theoretical approach. However, careful consideration as to which patients may benefit will be required and the outcome of clinical trials needed.

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“…In this regard, a phase 3 clinical trial is ongoing (NCT04570397). Ravulizumab has been shown to reduce complement-mediated inflammatory damage associated with COVID-19 infection in patients with paroxysmal nocturnal hemoglobinuria (PNH), according to a clinical case report study [123] . Annane et al suggested that Eculizumab leads to improved survival and reduced hypoxia in patients with severe COVID-19 [124] .…”

Section: Ravulizumab and Eculizumabmentioning

confidence: 99%

Complement system as the potential therapeutic target in the management of COVID-19 patients

et al. 2022

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The emerging COVID-19 caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has been raised as a global health challenge. Despite the breakthrough in the development of the COVID-19 vaccine, it still continues as a serious crisis, worldwide. The aberrant immune responses are strongly associated with the severity of the disease and an increased rate of morbidity and mortality among COVID-19 patients. The complement cascade activation is mediated by classical, lectin, and alternative pathways which could induce an inflammatory state during the COVID-19 infection. The growing body of research suggests that complement system activation plays an important role in the immunopathogenesis of SARS-CoV-2. Therefore, the blockade of complement cascades may be an effective approach to prevent the multi-organ complications of COVID-19. In this review, we will highlight the role of the complement system in the immunopathology of COVID-19, emphasizing the potential therapeutical targets to ameliorate COVID-19 infection.

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COVID19 infection in a patient undergoing treatment for Paroxysmal Nocturnal Hemoglobinuria (PNH) with Ravulizumab

Cited by 6 publications

References 16 publications

Complement Inhibition in Paroxysmal Nocturnal Hemoglobinuria (PNH): A Systematic Review and Expert Opinion from Central Europe on Special Patient Populations

Complement Inhibition in Paroxysmal Nocturnal Hemoglobinuria (PNH): A Systematic Review and Expert Opinion from Central Europe on Special Patient Populations

COVID19 infection in a patient with paroxysmal nocturnal hemoglobinuria

Complement system as the potential therapeutic target in the management of COVID-19 patients

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