• Yeow Tee Goh, Eng Soo Yap, Chuen Wen Tan, Daryl Tan, Yvonne Su Ming Loh, Yuh Shan Lee, Lip Leong Chong, Zi Yi Lim, and Hein Than.
• Department of Haematology, Singapore General Hospital, Singapore.
• Ann Acad Med Singap. 2024 Jun 28; 53 (6): 371385371-385.

IntroductionParoxysmal nocturnal haemoglobinuria (PNH) is a rare haematologic disease characterised by intravascular haemolysis, thrombophilia and bone marrow failure. There is a lack of established clinical guidance on the screening, diagnosis and manage-ment of PNH in Singapore. A relatively low level of awareness among healthcare professionals regarding PNH manifestations further contributes to diagnostic delays. Additionally, limited access to complement inhibitors, like eculizumab, may delay treatment and impact patient outcomes.MethodNine haematologists from different institu-tions in Singapore convened to formulate evidence-based consensus recommendations for optimising the diagnosis and management of patients with PNH and improving access to novel treatments. The experts reviewed the existing literature and international guidelines published from January 2010 to July 2023, focusing on 7 clinical questions spanning PNH screening, diagnostic criteria, investigations, treatment and monitoring of subclinical and classic disease, PNH with underlying bone marrow disorders, and PNH in pregnancy. A total of 181 papers were reviewed to formulate the statements. All experts voted on the statements via 2 rounds of Delphi and convened for an expert panel discussion to finetune the recommendations.ResultsSixteen statements have been formulated for optimising the screening, diagnosis and management of PNH. Upon confirmation of PNH diagnosis, individuals with active haemolysis and/or thrombosis should be considered for anti-complement therapy, with eculizumab being the only approved drug in Singapore.ConclusionThe current recommendations aim to guide the clinicians in optimising the screening, diagnosis and management of PNH in Singapore.

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