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Paroxysmal nocturnal hemoglobinuria

Última revisão: 13 Feb 2025
Última atualização: 19 Jan 2024

Resumo

Definição

História e exame físico

Principais fatores diagnósticos

  • hemoglobinuria
  • Budd-Chiari syndrome
  • thrombosis
Detalhes completos

Outros fatores diagnósticos

  • history of aplastic anemia
  • fatigue
  • anemia
  • abdominal pain
  • dysphagia and odynophagia
  • erectile dysfunction
  • dyspnea
  • infections
  • bleeding diathesis
  • neurologic signs and symptoms
Detalhes completos

Fatores de risco

  • aplastic anemia/hypoplastic bone marrow
  • myelodysplasia
Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

  • urine dipstick
  • urine microscopy
  • CBC
  • reticulocyte count
  • serum LDH
  • unconjugated bilirubin
  • D-dimer
Detalhes completos

Investigações a serem consideradas

  • haptoglobin
  • flow cytometry for glycosylphosphatidylinositol anchor or anchored proteins
  • fluorescent aerolysin
  • immunotyping
  • antiglobulin (Coombs) test
  • serum iron, serum ferritin
  • serum creatinine and BUN
Detalhes completos

Algoritmo de tratamento

AGUDA

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

CONTÍNUA

following acute clinical PNH with thrombosis: nonpregnant

Colaboradores

Autores

Ibrahim Ibrahim, MD

Associate Professor

Internal Medicine

UT Southwestern

Dallas

TX

Declarações

Taha Bat, MD

Assistant Professor

Internal Medicine

UT Southwestern

Dallas

TX

Declarações

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

Agradecimentos

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

利益声明

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

同行评议者

David Dingli, MD, PhD

Associate Professor of Medicine

Mayo Clinic

Rochester

MN

利益声明

DD declares that he has no competing interests.

Panayiotis D. Ziakas, MD, MSc, PhD

Department of Pathophysiology

Medical School

University of Athens

Athens

Greece

利益声明

PDZ declares that he has no competing interests.

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  • Guidelines

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