Diagnosing PNH
  • Bone marrow dysfunction10,11,14
  • Unprovoked and unexplained thrombosis10-12
  • Haemolysis (Any of: increased lactate dehydrogenase (LDH), decreased haptoglobin, increased reticulocyte count, increased bilirubin)10,11,14
  • Symptoms of anaemia (e.g. fatigue, tachycardia, shortness of breath, headache)11
  • Thrombosis11
  • Dark-coloured urine11
  • Intermittent abdominal pain11
  • Dysphagia11
  • Erectile dysfunction11
  • Abdominal and chest pain11,13
 Learn more about the symptoms of PNH
*Detects PNH cells down to 0.01% clone size.10 †Deep vein thrombosis and/or pulmonary embolism in a patient with no antecedent major clinical risk factor for venous thromboembolism that is not provoked by surgery, trauma, immobilization, hormonal therapy (oral contraceptive or hormone replacement therapy), or active cancer.11 ‡Unexplained persistent cytopenia in a patient in whom (minimal) diagnostic criteria for myelodyspastic syndrome are not fulfilled.11 §Anemia, neutropenia, or thrombocytepania.11,19 ∥Unusual sites include hepatic veins (Budd-Chiari syndrome), other intra-abdominal veins (portal, splenic, splanchnic), cerebral sinuses, and dermal veins.10A multicenter, prospective evaluation of diagnostic screening for PNH by flow cytometry in 3,938 peripheral blood samples tested in 24 laboratories in Spain (N=1,718) and a reference center in Brazil (N=2,220).18

Test for PNH with high-sensitivity flow cytometry with fluorescent aerolysin (FLAER) on peripheral blood, the gold-standard diagnostic test for PNH.20

If the test is positive: refer the patient to a PNH specialist.
Learn more
Adverse Event Reporting

Please report any adverse events via your national reporting system. Adverse events can also be reported to Alexion, AstraZeneca Rare Disease by contacting: https://contactazmedical.astrazeneca.com/
Kulasekararaj AG, et al. The Importance of Terminal Complement Inhibition in Paroxysmal Nocturnal Hemoglobinuria. Therapeutic Advances in Hematology. 2022;13:20406207221091046. Hill A. Paroxysmal nocturnal haemoglobinuria. Nature Reviews Disease Primers 2017. Jalbert JJ et al. Epidemiology of PNH an Real-World Treatment Patterns Following an Incident PNH Diagnosis in the US. Blood (2019) 134 (Supplement_1): 3407. Gembillo G, Siligato R, Cernaro V, et al. Complement Inhibition Therapy and Dialytic Strategies in Paroxysmal Nocturnal Hemoglobinuria: the Nephrologist’s Opinion. Journal of Clinical Medicine 2020;9:1261.  Schrezenmeier H, et al. Baseline Characteristics and Disease Burden in Patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry. Haematologica. 2014;99:922–929. Brodsky RA, Paroxysmal Nocturnal Hemoglobinuria, Hematology: Basic Principles and Practice. Elsevier Inc. 2018. Kulagin AD, et al. Paroxysmal nocturnal hemoglobinuria in children and adults: comparative clinical profile and long-term prognosis. Pediatric Hematology/Oncology And Immunopathology. 2018;17(3), 11-21. Sharma V R. Paroxysmal Nocturnal Hemoglobinuria: Pathogenesis, Testing and Diagnosis. Clinical Advances in Hematology and Oncology. 2013. Mitchell R, et al. Path to Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: The Results of an Exploratory Study Conducted by the Aplastic Anemia and MDS International Foundation and the National Organization for Rare Disorders Utilizing an Internet-Based Survey. SM Clinical Medical Oncology. 2017;1:1001. Borowitz MJ, Craig FE, Digiuseppe JA, et al. Guidelines for the Diagnosis and Monitoring of Paroxysmal Nocturnal Hemoglobinuria and Related Disorders by Flow Cytometry. Cytometry Part B Clinical Cytometry. 2010;78(4):211-30. Roth A, Maciejewski J, Nishimura JI, et al. Screening and Diagnostic Clinical Algorithm for Paroxysmal Nocturnal Hemoglobinuria: Expert Consensus. European Journal of Haematology. 2018;101(1):3-11. Hillmen P, Elebute M, Kelly R, et al. Long-term Effect of the Complement Inhibitor Eculizumab on Kidney Function in Patients with Paroxysmal Nocturnal Hemoglobinuria. American Journal of Hematology. 2010;85(8):553-9. Sahin F, Akay OM, Ayer M, et al. Pesg PNH Diagnosis, Follow-up and Treatment Guidelines. American Journal of Blood Research. 2016;6(2):19-27. Parker C, Omine M, Richards S, et al. Diagnosis and Management of Paroxysmal Nocturnal Hemoglobinuria. Blood. 2005;106(12):3699-709. Brodsky RA. Paroxysmal Nocturnal Hemoglobinuria. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 4th ed. Elsevier Churchill Livingstone; 2005:419-427. Canadian PNH Network. Published 2021. Accessed November 2024. Available here. Dezern AE and Borowitz MJ. ICCS/ESCCA Consensus Guidelines to Detect GPI-deficient Cells in Paroxysmal Nocturnal Hemoglobinuria (PNH) and Related Disorders Part 1 - Clinical Utility. Cytometry Part B: Clinical Cytometry. 2018;94:16–22. Morado M, et al. Diagnostic Screening of Paroxysmal Nocturnal Hemoglobinuria: Prospective Multicentric Evaluation of the Current Medical Indications. Cytometry Part B; Clinical Cytometry. 2017; 92B:361–370. Seidel MG. Autoimmune and Other Cytopenias in Primary Immunodeficiencies: Pathomechanisms, Novel Differential Diagnoses, and Treatment. Blood. 2014;124(15):2337-44. Szlendak U. Paroxysmal Nocturnal Hemoglobinuria: Advances in the Understanding of Pathophysiology, Diagnosis, and Treatment. Polish Articles of Internal Medicine. 2022. advance I am a healthcare professional registered in the EU I am not a
healthcare professional