{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:01:19Z","timestamp":1760144479993,"version":"build-2065373602"},"reference-count":17,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2024,4,19]],"date-time":"2024-04-19T00:00:00Z","timestamp":1713484800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematology Reports"],"abstract":"<jats:p>The co-occurrence of myasthenia gravis (MG) and paroxysmal nocturnal hemoglobinuria (PNH) is rare; only one case has been published so far. We report a 63-year-old Caucasian female patient who was diagnosed with MG at the age of 43. Thymoma was also detected, and so it was surgically resected, which resulted in reasonable disease control for nearly 20 years. Slight hemolysis began to emerge, and then myasthenia symptoms progressed, so immunosuppressive therapy was started. Due to progressive disease and respiratory failure, the patient underwent plasmapheresis, and ventilatory support was stopped. Marked hemolysis was present, and diagnostic tests confirmed PNH with type III PNH cells. Her myasthenia symptoms aggravated, mechanical ventilation had to be started again, and due to the respiratory acidosis, massive hemolysis occurred. After two plasmapheresis sessions, the patient received eculizumab at 600 mg, resulting in prompt hemolysis control. After the second dose of the treatment, the patient was extubated. Still, due to their inability to cough, she developed another respiratory failure and pneumonia\u2013sepsis, resulting in the patient\u2019s death. This case highlights the rare association between these two serious diseases and similar immune-mediated pathophysiology mechanisms involving the complement system.<\/jats:p>","DOI":"10.3390\/hematolrep16020025","type":"journal-article","created":{"date-parts":[[2024,4,19]],"date-time":"2024-04-19T06:28:09Z","timestamp":1713508089000},"page":"255-259","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Eculizumab Treatment of Massive Hemolysis Occurring in a Rare Co-Existence of Paroxysmal Nocturnal Hemoglobinuria and Myasthenia Gravis"],"prefix":"10.3390","volume":"16","author":[{"given":"R\u00e1hel R\u00e9ka","family":"Bicsk\u00f3","sequence":"first","affiliation":[{"name":"Department of Hematology, Institute of Internal Medicine, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c1rp\u00e1d","family":"Ill\u00e9s","sequence":"additional","affiliation":[{"name":"Department of Hematology, Institute of Internal Medicine, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7364-9906","authenticated-orcid":false,"given":"Zsuzsanna","family":"Hevessy","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gergely","family":"Iv\u00e1dy","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gy\u00f6rgy","family":"Kerekes","sequence":"additional","affiliation":[{"name":"Intensive Care Unit, Institute of Internal Medicine, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00e1bor","family":"M\u00e9hes","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8305-3209","authenticated-orcid":false,"given":"T\u00fcnde","family":"Cs\u00e9p\u00e1ny","sequence":"additional","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4092-5465","authenticated-orcid":false,"given":"Lajos","family":"Gergely","sequence":"additional","affiliation":[{"name":"Department of Hematology, Institute of Internal Medicine, Faculty of Medicine, Clinical Center, University of Debrecen, 4032 Debrecen, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,4,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"F1000 Faculty Rev-1513","DOI":"10.12688\/f1000research.8206.1","article-title":"Pathogenesis of myasthenia gravis: Update on disease types, models, and mechanisms","volume":"5","author":"Phillips","year":"2016","journal-title":"F1000Research"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1643","DOI":"10.1007\/s00415-019-09498-4","article-title":"Role of complement and potential of complement inhibitors in myasthenia gravis and neuromyelitis optica spectrum disorders: A brief review","volume":"268","author":"Chamberlain","year":"2021","journal-title":"J. Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"917","DOI":"10.3389\/fimmu.2020.00917","article-title":"Complement Inhibitor Therapy for Myasthenia Gravis","volume":"11","author":"Albazli","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1016\/S1474-4422(17)30369-1","article-title":"Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): A phase 3, randomised, double-blind, placebo-controlled, multicentre study","volume":"16","author":"Howard","year":"2017","journal-title":"Lancet Neurol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1398","DOI":"10.1002\/acn3.51376","article-title":"REGAINSG Long-term efficacy of eculizumab in refractory generalized myasthenia gravis: Responder analyses","volume":"8","author":"Howard","year":"2021","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1001\/jamaneurol.2019.5125","article-title":"Clinical Effects of the Self-administered Subcutaneous Complement Inhibitor Zilucoplan in Patients With Moderate to Severe Generalized Myasthenia Gravis: Results of a Phase 2 Randomized, Double-Blind, Placebo-Controlled, Multicenter Clinical Trial","volume":"77","author":"Howard","year":"2020","journal-title":"JAMA Neurol."},{"key":"ref_7","first-page":"S3","article-title":"Paroxysmal nocturnal hemoglobinuria: Role of the complement system, pathogenesis, and pathophysiology","volume":"26","author":"Bektas","year":"2020","journal-title":"J. Manag. Care Spec. Pharm."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"3223","DOI":"10.1038\/s41375-021-01190-9","article-title":"Clinical and prognostic significance of small paroxysmal nocturnal hemoglobinuria clones in myelodysplastic syndrome and aplastic anemia","volume":"35","author":"Fattizzo","year":"2021","journal-title":"Leukemia"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1111\/bjh.17147","article-title":"Paroxysmal nocturnal haemoglobinuria (PNH): Novel therapies for an ancient disease","volume":"191","author":"Luzzatto","year":"2020","journal-title":"Br. J. Haematol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"E1","DOI":"10.1002\/ajh.26354","article-title":"Upfront unrelated donor hematopoietic stem cell transplantation in patients with idiopathic aplastic anemia: A retrospective study of the Severe Aplastic Anemia Working Party of European Bone Marrow Transplantation. 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Insights Oncol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1002\/cyto.b.21609","article-title":"ICCS\/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 3\u2014Data analysis, reporting and case studies","volume":"94","author":"Illingworth","year":"2018","journal-title":"Cytom. B Clin Cytom."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1080\/08830185.2020.1833877","article-title":"Complement system network in cell physiology and in human diseases","volume":"40","author":"Romano","year":"2021","journal-title":"Int. Rev. Immunol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"577767","DOI":"10.1016\/j.jneuroim.2021.577767","article-title":"Eculizumab treatment for myasthenia gravis subgroups: 2021 update","volume":"362","author":"Jiao","year":"2021","journal-title":"J. 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