Abstract
Introduction: paroxysmal nocturnal hemoglobinuria (PNH) is a very rare acquired disorder of hematopoietic stem cells and a life threatening disease ; characterized by compliment mediated hemolytic anemia, thrombosis and impaired bone marrow function. There are only over 4000 patients in the international PNH registry. In this report we will describe a case of a Saudi male patient diagnosed with PNH.
Case report: A 37 year old male patient presented to our hospital (King Fahad Hospital Hufof) with shortness of breath, palpitation and fatigability; on a background history of multiple prior presentations with red urine. His investigations showed non autoimmune hemolytic anemia and leucopenia . After an extensive work-up, he was found to have paroxysmal nocturnal hemoglobinuria with Aplastic Anemia which requires careful evaluation and management as it is associated with morbidity and mortality if not treated properly. Conclusion: Physicians needs high index of suspicion for the diagnosis of PNH to avoid serious complications of the disease.