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Year : 2014  |  Volume : 5  |  Issue : 2  |  Page : 37-44

Paroxysmal nocturnal hemoglobinuria: Diagnosis and management protocol


1 Department of Hematology and Oncology, King Saud University Medical City, Riyadh, Saudi Arabia
2 Departments of Hematology and Pathology, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia
3 Departments of Oncology, King Abdulaziz Medical City, Jeddah, Saudi Arabia
4 Department of Hematology, Dubai Hospital, Dubai, Saudi Arabia
5 Department of Oncology, King Abdulaziz Medical City, Riyadh, Saudi Arabia
6 Department of Pathology and Hematology, Mubarak Hospital, Kuwait City, Kuwait
7 Department of Hematology and Pathology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
8 Department of Hematology, Dubai Hospital, Dubai, United Arab Emirates
9 Department of Hematology, College of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
10 Department of Pathology, Rashid Hospital, Dubai, United Arab Emirates
11 Department of Hematology, Qabous University Hospital, Mascut, Oman
12 Department of Hematology, Security Forces Hospital, Riyadh, Saudi Arabia

Correspondence Address:
Abdul Kareem Almomen
Center of Excellence in Thrombosis and Hemostasis, King Saud University Medical City, P. O. Box: 66533, Riyadh 11586
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-5127.137081

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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired, rare clonal blood disorder, characterized by chronic intravascular hemolysis, bone marrow failure, renal failure and pulmonary hypertension, and a heightened risk of thrombotic complications. PNH etiology is an Υ-linked gene somatic mutation of the phosphatidylinositol glycan class ΐ (PIG-A ), that results in deficiency of the glycosylphosphatidylinositol anchor structure responsible for fixing a wide spectrum of proteins on blood cell membranes, absence of these proteins, particularly CD55 and CD59, dysregulates the complement on cell membranes and results in significant chronic complement-mediated hemolysis. Early diagnosis of PNH is crucial for effective disease management. However, the heterogeneity of clinical symptoms and rarity of this disease usually results in untimely diagnosis, severe disability of patients, and increased risk of fatal complication. These recommendations are formulated by a panel of experts from the gulf cooperation countries. This information reflects their experience and to assist specialists looking after PNH patients, including hematologists, nephrologists, dialysis specialists, gastroenterologists, cardiologists, and surgeons.


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