Mar, 2016 16 hemoglobinuria paroxistica nocturna dra diana otero. The molecular basis of paroxysmal nocturnal hemoglobinuria. Dolores fraga fuentes, juan carlos valenzuela gamez. Paroxysmal nocturnal haemoglobinuria pnh is a clinical mani festation of an haemathology cell disease, whose etiology has been unk nown for many years. Paroxysmal nocturnal hemoglobinuria pnh is a rare, acquired, lifethreatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the bodys innate immune system. Existem tres caminhos pelos quais o complemento e ativado. Paroxysmal nocturnal hemoglobinuria affects both sexes equally, and can occur at any age, although it is most often diagnosed in young adulthood. Apresentase frequentemente com infeccoes recorrentes, neutropenia e trombocitopenia, e surge em associacao com outras doencas. This classification was abandoned because of the observation that surface proteins were missing not only in the rbc membrane but also in all blood cells, including the platelet and white cells. Eculizumab and allogeneic bone marrow transplantation is the only effective therapies. Arango elkin fernando, arroyave claudia lucia, zuleta john jairo, restrepo maria victoria, lopera antonio jose, zuluaga hector, botero juan pablo, zapata silvia. Jan 02, 2019 paroxysmal nocturnal hemoglobinuria pnh was previously classified as purely an acquired hemolytic anemia due to a hematopoietic stem cell mutation defect. This destructive process occurs due to the presence of defective surface protein daf on the red blood cell, which normally functions to inhibit such immune. Pnh results in the death of approximately 50% of affected individuals due to thrombotic complications and, until recently, had no specific therapy.
The paroxysmal nocturnal hemoglobinuria is an infrequent cause of pancytopenia. Hemoglobinuria paroxistica nocturna by prezi user on. Download as pptx, pdf, txt or read online from scribd. Hemoglobinuria paroxistica noturna diagnostico e tratamento. Hemoglobinuria paroxistica nocturna sistema complementario. Paroxysmal nocturnal hemoglobinuria internal medicine usmle step 2.
Hemoglobinuria paroxistica nocturna linkedin slideshare. Hemoglobinuria paroxistica noturna dos sintomas ao. Fisiopatologia e diagnostico da hemoglobinuria paroxistica. Natural history of paroxysmal nocturnal hemoglobinuria. Sintomas hemoglobinuria paroxistica nocturna medicinatv. This destructive process occurs due to the presence of defective surface protein daf on the red blood cell, which normally. Principais sintomas da hemoglobinuria paroxistica noturna e. Paroxysmal nocturnal hemoglobinuria pnh is a rare, chronic, debilitating disorder that most frequently presents in early adulthood and usually continuous throughout the life of the patient. Paroxysmal nocturnal hemoglobinuria pnh is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis. Hemoglobinuria paroxistica nocturna hematologia method of choice for diagnosis. Oct 30, 2014 paroxysmal nocturnal hemoglobinuria pnh is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis. Hemoglobinuria paroxistica nocturna infobioquimica.
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