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math113keybookdownloadRecent Epidemiological Studies of Paroxysmal Nocturnal Hemoglobinuria in the United States.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematologic disorder caused by somatic mutation of a gene that promotes dyserythropoiesis leading to bone marrow failure and hemolysis. PNH is a rare condition with a lifetime risk of venous thromboembolism (VTE) of 13% and a documented 2.6-fold higher risk of arterial thrombosis compared with the general population. In the International PNH Registry (IPRH), VTE was reported at a median age of 36 years in patients with PNH with a median follow-up of 52 months. The hematology practice of Dr. Dawson describes 3 different patients who presented in the last 12 years and highlights the potential differential diagnosis of PNH in patients who present with thrombosis and hemolysis. While PNH is not known to cause clinically significant arterial thrombosis in the general population, PNH patients can present with arterial thrombosis that appears atypical to other causes of arterial occlusion, such as atrial fibrillation, scleroderma, or prothrombotic conditions. The PNH-specific issues associated with recurrent venous thrombosis include optimizing medical anticoagulation during periods of hemolysis and the importance of prophylactic low molecular weight heparin and warfarin use for the primary prevention of VTE recurrence in these patients.Mount Pleasant (TN) County
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