Paroxysmal nocturnal hemoglobinuria
Resumen
Определение
Анамнез и осмотр
Ключевые диагностические факторы
- hemoglobinuria
- Budd-Chiari syndrome
- thrombosis
Другие диагностические факторы
- history of aplastic anemia
- fatigue
- anemia
- abdominal pain
- dysphagia and odynophagia
- erectile dysfunction
- dyspnea
- infections
- bleeding diathesis
- neurologic signs and symptoms
Факторы риска
- aplastic anemia/hypoplastic bone marrow
- myelodysplasia
Диагностические исследования
Исследования, которые показаны в первую очередь
- urine dipstick
- urine microscopy
- CBC
- reticulocyte count
- serum LDH
- unconjugated bilirubin
- D-dimer
Исследования, проведение которых нужно рассмотреть
- haptoglobin
- flow cytometry for glycosylphosphatidylinositol anchor or anchored proteins
- fluorescent aerolysin
- immunotyping
- antiglobulin (Coombs) test
- serum iron, serum ferritin
- serum creatinine and BUN
Алгоритм лечения
classic PNH (hemolytic anemia): nonpregnant
classic PNH (hemolytic anemia): pregnant
PNH in the setting of another specific bone marrow disorder: nonpregnant
PNH in the setting of another specific bone marrow disorder: pregnant with thrombosis
PNH in the setting of another specific bone marrow disorder: pregnant without thrombosis
subclinical PNH: nonpregnant
subclinical PNH: pregnant
following acute clinical PNH with thrombosis: nonpregnant
Составители
Авторы
Ibrahim Ibrahim, MD
Associate Professor
Internal Medicine
UT Southwestern
Dallas
TX
Раскрытие информации
II declares that he has no competing interests.
Taha Bat, MD
Assistant Professor
Internal Medicine
UT Southwestern
Dallas
TX
Раскрытие информации
TB serves on advisory boards for Alexion Pharmaceuticals and Novartis. He is also the author of an article cited in this topic.
Выражение благодарностей
Dr Ibrahim and Dr Bat would like to gratefully acknowledge Dr Ilene Ceil Weitz and Dr Wendell F. Rosse, previous contributors to this topic.
Раскрытие информации
WFR serves on advisory boards and educational faculties for Alexion Pharmaceuticals. ICW receives speaker honoraria from and undertakes consultancy for Alexion Pharmaceuticals.
Рецензенты
David Dingli, MD, PhD
Associate Professor of Medicine
Mayo Clinic
Rochester
MN
Раскрытие информации
DD declares that he has no competing interests.
Panayiotis D. Ziakas, MD, MSc, PhD
Department of Pathophysiology
Medical School
University of Athens
Athens
Greece
Раскрытие информации
PDZ declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
Список литературы
Основные статьи
Parker CJ. Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria. ASH Education Book 2016;2016(1):208-16.Полный текст Аннотация
National Organization for Rare Disorders. NORD rare disease report: paroxysmal nocturnal hemoglobinuria. May 2024 [internet publication].Полный текст
Dezern AE, Borowitz MJ. ICCS/ESCCA Consensus Guidelines to detect GPI‐deficient cells in Paroxysmal Nocturnal Hemoglobinuria (PNH) and related Disorders Part 1 – Clinical Utility. Cytometry B Clin Cytom. 2018;94(1):16-22.Полный текст Аннотация
Статьи, указанные как источники
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Autoimmune hemolytic anemia, warm antibody type
- Microangiopathic hemolytic anemia
- Paroxysmal cold hemoglobinuria
Больше DifferentialsGuidelines
- Paroxysmal nocturnal hemoglobinuria
- Consensus guidelines to detect GPI‐deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders: Part 1 – clinical utility
Больше GuidelinesPatient information
Deep vein thrombosis
High blood pressure
More Patient information
Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer