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Hemoglobinúria paroxística noturna

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 20 Aug 2025
Last updated: 19 Mar 2025

Summary

Definition

History and exam

Key diagnostic factors

  • hemoglobinúria
  • Síndrome de Budd-Chiari
  • trombose
Full details

Other diagnostic factors

  • história de anemia aplásica
  • fadiga
  • anemia
  • dor abdominal
  • disfagia e odinofagia
  • disfunção erétil
  • dispneia
  • infecções
  • diátese hemorrágica
  • sinais e sintomas neurológicos
Full details

Risk factors

  • anemia aplásica/hipoplásica da medula óssea
  • mielodisplasia
Full details

Diagnostic tests

1st tests to order

  • tira reagente para exame de urina
  • microscopia da urina
  • Hemograma completo
  • contagem de reticulócitos
  • lactato desidrogenase (LDH) sérico
  • bilirrubina não conjugada
  • dímero D
Full details

Tests to consider

  • haptoglobina
  • citometria de fluxo para âncora de glicosilfosfatidilinositol ou proteínas ancoradas
  • aerolisina fluorescente
  • imunofenotipagem
  • teste de antiglobulina (Coombs)
  • ferro e ferritina séricos
  • creatinina sérica e ureia
Full details

Treatment algorithm

ACUTE

hemoglobinúria paroxística noturna (HPN) clássica (anemia hemolítica): não gestante

hemoglobinúria paroxística noturna (HPN) clássica (anemia hemolítica): gestante

HPN no contexto de outro distúrbio de medula óssea específico: não gestante

HPN no contexto de outro distúrbio de medula óssea específico: gestante com trombose

HPN no contexto de outro distúrbio de medula óssea específico: gestante sem trombose

HPN subclínica: não gestante

HPN subclínica: gestante

ONGOING

após HPN clínica aguda com trombose: não gestante

Contributors

Authors

Ibrahim Ibrahim, MD

Associate Professor

Internal Medicine

UT Southwestern

Dallas

TX

Disclosures

II declares that he has no competing interests.

Taha Bat, MD

​Assistant Professor

Internal Medicine

UT Southwestern

Dallas

TX

Disclosures

TB serves on advisory boards for Alexion Pharmaceuticals and Novartis. He is also the author of an article cited in this topic.

Acknowledgements

Dr Ibrahim and Dr Bat would like to gratefully acknowledge Dr Ilene Ceil Weitz and Dr Wendell F. Rosse, previous contributors to this topic.

Disclosures

WFR serves on advisory boards and educational faculties for Alexion Pharmaceuticals. ICW receives speaker honoraria from and undertakes consultancy for Alexion Pharmaceuticals.

Peer reviewers

David Dingli, MD, PhD

Associate Professor of Medicine

Mayo Clinic

Rochester

MN

Disclosures

DD declares that he has no competing interests.

Panayiotis D. Ziakas, MD, MSc, PhD

Department of Pathophysiology

Medical School

University of Athens

Athens

Greece

Disclosures

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.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Parker CJ. Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria. ASH Education Book 2016;2016(1):208-16.Full text  Abstract

National Organization for Rare Disorders. NORD rare disease report: paroxysmal nocturnal hemoglobinuria. May 2024 [internet publication].Full text

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.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

  • Differentials

    • Anemia hemolítica autoimune por anticorpos a quente (warm antibody type)
    • Anemia hemolítica microangiopática
    • Hemoglobinúria paroxística ao frio
    More Differentials

  • Guidelines

    • Paroxysmal nocturnal hemoglobinuria
    • Consensus guidelines to detect GPI‐deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders: Part 1 – clinical utility
    More Guidelines

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