COVID19 infection in a patient with paroxysmal nocturnal hemoglobinuria: A case report.
Adult
Anticoagulants
/ administration & dosage
COVID-19
/ diagnosis
COVID-19 Serological Testing
Complement Activation
/ drug effects
Complement Inactivating Agents
/ administration & dosage
Hemoglobinuria, Paroxysmal
/ blood
Humans
Male
SARS-CoV-2
/ immunology
Symptom Flare Up
Thrombosis
/ immunology
Treatment Outcome
COVID-19 Drug Treatment
Journal
Medicine
ISSN: 1536-5964
Titre abrégé: Medicine (Baltimore)
Pays: United States
ID NLM: 2985248R
Informations de publication
Date de publication:
21 May 2021
21 May 2021
Historique:
received:
30
11
2020
accepted:
18
03
2021
entrez:
20
5
2021
pubmed:
21
5
2021
medline:
27
5
2021
Statut:
ppublish
Résumé
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. 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. The patient was diagnosed with acute hemolytic exacerbation of PNH complicated with moderate COVID19 pneumonia. The patient was initiated with an anticoagulant unfractionated heparin, dexamethasone, and cefuroxime injection. His symptoms quickly resolved, and he was discharged after 5 days. 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.
Identifiants
pubmed: 34011023
doi: 10.1097/MD.0000000000025456
pii: 00005792-202105210-00004
pmc: PMC8137037
doi:
Substances chimiques
Anticoagulants
0
Complement Inactivating Agents
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e25456Subventions
Organisme : Agentúra Ministerstva Školstva, Vedy, Výskumu a Športu SR
ID : APVV-17-0054
Organisme : Vedecká Grantová Agentúra MŠVVaŠ SR a SAV
ID : VEGA 1/0187/17
Informations de copyright
Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.
Déclaration de conflit d'intérêts
The authors have no conflicts of interests to disclose.
Références
Risitano AM, Rotoli B. Paroxysmal nocturnal hemoglobinuria: pathophysiology, natural history and treatment options in the era of biological agents. Biologics 2008;2:205–22.
Li G, Hu R, Gao Y. Acute myocardial infarction in a Chinese patient with paroxysmal nocturnal hemoglobinuria: a case report. Medicine (Baltimore) 2019;98:e16657.
Bastos JMC, Pinheiro PL, Rocha LC, et al. Therapeutic challenges in pregnant women with paroxysmal nocturnal hemoglobinuria: a case report. Medicine (Baltimore) 2018;97:e12155.
de Brito Junior LC, Cardoso Mdo S, Rocha EG, et al. Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil. Rev Bras Hematol Hemoter 2011;33:35–7.
Alencar RCB, Guimarães AM, Brito Junior LC. Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant. J Bras Patol Med Lab 2016;52:307–11.
Devalet B, Mullier F, Chatelain B, et al. Pathophysiology, diagnosis, and treatment of paroxysmal nocturnal hemoglobinuria: a review. Eur J Haematol 2015;95:190–8.
Sutherland DR, Keeney M, Illingworth A. Practical guidelines for the high-sensitivity detection and monitoring of paroxysmal nocturnal hemoglobinuria clones by flow cytometry. Cytometry B Clin Cytom 2012;82:195–208.
Höchsmann B, Rojewski M, Schrezenmeier H. Paroxysmal nocturnal hemoglobinuria (PNH): higher sensitivity and validity in diagnosis and serial monitoring by flow cytometric analysis of reticulocytes. Ann Hematol 2011;90:887–99.
Rosti V. The molecular basis of paroxysmal nocturnal hemoglobinuria. Haematologica 2000;85:82–7.
Gembillo G, Siligato R, Cernaro V, et al. Complement inhibition therapy and dialytic strategies in paroxysmal nocturnal hemoglobinuria: the nephrologist's opinion. J Clin Med 2020;9:1261.
Shikdar S, Borogovac A, Mohamad E, et al. COVID19 infection in a patient undergoing treatment for paroxysmal nocturnal hemoglobinuria (PNH) with ravulizumab. Res Squere 2020;1:01–6.
Bosmann M. Complement activation during critical illness: current findings and an outlook in the era of COVID-19. Am J Respir Crit Care Med 2020;202:163–5.
Fletcher-Sandersjöö A, Bellander BM. Is COVID-19 associated thrombosis caused by overactivation of the complement cascade? A literature review. Thromb Res 2020;194:36–41.
Kulasekararaj AG, Lazana I, Large J, et al. Terminal complement inhibition dampens the inflammation during COVID-19. Br J Haematol 2020;190:e141–3.
Risitano AM, Mastellos DC, Huber-Lang M, et al. Complement as a target in COVID-19? Nat Rev Immunol 2020;20:343–4.
Schüller H, Klein F, Lübbert M, et al. Hemolytic crisis in a patient treated with eculizumab for paroxysmal nocturnal hemoglobinuria possibly triggered by SARS-CoV-2 (COVID-19): a case report. Ann Hematol 2021;100:841–2.
Pike A, Muus P, Munir T, et al. COVID-19 infection in patients on anti-complement therapy: the Leeds National Paroxysmal Nocturnal Haemoglobinuria service experience. Br J Haematol 2020;191:e1–4.
Araten DJ, Belmont HM, Schaefer-Cutillo J, et al. Mild clinical course of COVID-19 in 3 patients receiving therapeutic monoclonal antibodies targeting C5 complement for hematologic disorders. Am J Case Rep 2020;21:e927418.
Genthon A, Chiarabini T, Baylac P, et al. Severe COVID-19 infection in a patient with paroxysmal nocturnal hemoglobinuria on eculizumab therapy. Leuk Lymphoma 2021;8:01–4.