Last reviewed: 28 Sep 2021
Last updated: 20 Sep 2018

Summary

Definition

History and exam

Key diagnostic factors

  • hemoglobinuria
  • Budd-Chiari syndrome
  • thrombosis

Other diagnostic factors

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

Risk factors

  • aplastic anemia/hypoplastic bone marrow
  • myelodysplasia

Diagnostic investigations

1st investigations to order

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

Investigations to consider

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

Treatment algorithm

Contributors

Authors

Ilene Ceil Weitz, MD

Associate Professor of Clinical Medicine

Jane Anne Nohl Division of Hematology

Keck-USC School of Medicine

Los Angeles

CA

Disclosures

ICW receives speaker honoraria from and undertakes consultancy for Alexion Pharmaceuticals.

Acknowledgements

Dr Ilene Ceil Weitz would like to gratefully acknowledge Dr Wendell F. Rosse, the previous contributor to this topic. WFR serves on advisory boards and educational faculties 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.

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