Screening and diagnostic clinical algorithm for paroxysmal nocturnal hemoglobinuria: Expert consensus
Alexander Röth1, Jaroslaw Maciejewski2, Jun-Ichi Nishimura3
1University Hospital Essen, Essen, Germany.
European Journal of Haematology
|March 14, 2018
Summary
Developing a screening and diagnostic algorithm for paroxysmal nocturnal hemoglobinuria (PNH) aids non-specialists. This consensus-driven tool improves early identification of this rare, life-threatening blood disorder.
Area of Science:
- Hematology
- Clinical diagnostics
Background:
- Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, severe blood disorder with non-specific symptoms, often leading to diagnostic delays.
- Existing hematologic guidelines lack international expert consensus and actionable algorithms for non-specialist PNH screening and diagnosis.
Purpose of the Study:
- To develop a clinically relevant, consensus-driven screening and diagnostic algorithm for paroxysmal nocturnal hemoglobinuria (PNH).
- To provide non-specialist clinicians with actionable guidance to facilitate early PNH identification and diagnosis.
Main Methods:
- A modified Delphi methodology was employed with an international expert advisory committee of PNH specialists.
- Twelve global experts evaluated 5 blinded case studies through two rounds of online questionnaires to reach consensus on diagnostic approaches.
Main Results:
- Consensus was achieved on 95% of screening and diagnostic decision points within the algorithm.
- Agreement was reached on 90% of the tests required at various decision points in the diagnostic pathway.
Conclusions:
- A consensus-based algorithm for PNH screening and diagnosis was successfully developed.
- The algorithm offers practical guidance for non-specialist clinicians, aiming to improve the timely diagnosis of PNH.
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