Early diagnosis is essential for improved patient management and prognosis. Learn about ICCS and other expert recommendations for who should be tested for PNH.
Early diagnosis is essential for improved patient management and prognosis2,3
The devastating nature of PNH demands early diagnosis and intervention1-3
The International Clinical Cytometry Society (ICCS) has devised guidelines to help in the diagnosis of patients with PNH.2
The 2010 ICCS Guidelines identify patient populations at high-risk for PNH2
- Routinely evaluate high-risk patient types for PNH: these include patients with unexplained cytopenias,
aplastic anemia, myelodysplastic syndromes, unexplained thrombosis, Coombs-negative hemolytic anemia, and hemoglobinuria2,4,7-11
- ICCS Guidelines recommend high-sensitvity (0.01% PNH cell threshold) flow cytometry testing on patients at high risk for PNH2
- Early diagnosis and intervention are critical2,3
A comprehensive clinical assessment is crucial to determine the risk for morbidities and premature mortality in your patient with PNH4,5,12,14,15
- PNH presents uniquely in each patient, and outward symptomatology may be general and common2
- No one sign or symptom provides a diagnosis of PNH4
- Rule PNH in or out using high-sensitivity flow cytometry on a peripheral blood sample2
- Appropriate clinical assessment of the patient along with lab results provide a more complete diagnostic picture4,12-15
Monitoring of patients with small PNH clones is essential since clone size increased in 40% (10/25) patients with PNH clone size between 0.11% and 10%6
*IPIG = International PNH Interest Group.
References: 1. Hillmen P, Lewis SM, Bessler M, et al. N Engl J Med. 1995;333:1253-1258. 2. Borowitz MJ, Craig FE, DiGiuseppe JA, et al; for Clinical Cytometry Society. Cytometry Part B. 2010;78B:211-230. 3. Richards SJ, Barnett D. Clin Lab Med. 2007;27:577-590. 4. Parker C, Omine M, Richards S, et al; for International PNH Interest Group. Blood. 2005;106:3699-3709. 5. Weitz I, Meyers G, Lamy T, et al. Cross-sectional validation study of patient-reported outcomes in patients with paroxysmal nocturnal haemoglobinuria. Intern Med J. 2013;43:298-307. 6. Movalia M, lllingworth A, Weitz I, et al. Poster presented at the 53rd Annual Meeting of the American Society of Hematology; December 10-13, 2011; San Diego, CA. Abstract 1033. 7. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Myelodysplastic syndromes. Version 2.2014. http://www.nccn.org/professionals/physician_gls/pdf/mds. Accessed February 10, 2014. 8. Lee JW, Jang JH, Lee JH, et al. Haematologica.2010;95(s2):205-206. 9. Hill A, Kelly R, Hillmen P. Blood. 2013;121:4985-4996. 10. Mohanty BD, De Castro CM. Am J Med. 2012;125:243-245. 11. Urbano-Ispizua A, Schrezenmeier H, Brodsky R, et al. Evaluation of paroxysmal nocturnal hemoglobinuria disease burden in patients enrolled in the International PNH Registry. In: Abstracts of the 15th Congress of the European Hematology Association; June 10-13, 2010; Barcelona, Spain. Abstract 1022. 12. Hill A, Rother RP, Wang X, et al. Br J Haematol. 2010;149:414-425. 13. Meyers G, Weitz I, Lamy T, et al. Blood. 2007;110: Abstract 3683. 14. Nishimura J-I, Kanakura Y, Ware RE, et al. Medicine. 2004;83:193-207. 15. Hill A, Richards SJ, Hillmen P. Br J Haematol. 2007;137:181-192. 16. Rother RP, Bell L, Hillmen P, et al. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA. 2005;293:1653-1662.