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Post a Comment Difficulty of diagnostics and efficiency of pathogenetic therapy of paroxysmal nocturnal hemoglobinuria (clinical case)
- Authors: Barlamov P.N.1, Vasilyeva E.R.2, Zholobov V.G.1
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Affiliations:
- E.A. Vagner Perm State Medical University
- City Clinical Polyclinic
- Issue: Vol 40, No 3 (2023)
- Pages: 131-138
- Section: Clinical case
- URL: https://ogarev-online.ru/PMJ/article/view/254872
- ID: 254872
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Abstract
To show the features of the clinical picture of this orphan disease and demonstrate the effectiveness of a long-term pathogenetic treatment of this disease using the case history of a patient with paroxysmal nocturnal hemoglobinuria (PNH) as an example. Complaints, anamnesis, physical status, as well as laboratory and instrumental studies used for a patient with PNH were presented. Modern literature data on the diagnosis and treatment of PNH have been studied.
Complaints, anamnesis, physical status as well as laboratory and instrumental studies used for PNH patient were analyzed in comparison with the world literature data on this pathology.
A conclusion was made regarding the need for a wide coverage of the clinical picture and diagnosis of PNH. The effectiveness of a long-term (for 13 years) pathogenetic treatment of the classical form of PNH with eculizumab was shown.
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##article.viewOnOriginalSite##About the authors
P. N. Barlamov
E.A. Vagner Perm State Medical University
Author for correspondence.
Email: pavel-barlamov@yandex.ru
ORCID iD: 0000-0002-8704-692X
MD, PhD, Professor, Associate Professor
Russian Federation, PermE. R. Vasilyeva
City Clinical Polyclinic
Email: pavel-barlamov@yandex.ru
ORCID iD: 0000-0002-0267-9729
hematologist
Russian Federation, PermV. G. Zholobov
E.A. Vagner Perm State Medical University
Email: pavel-barlamov@yandex.ru
ORCID iD: 0000-0003-0780-3116
MD, PhD, Professor
Russian Federation, PermReferences
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