Hemoglobinuria paroxistica nocturna pdf file download

Download as pptx, pdf, txt or read online from scribd. 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. Hemoglobinuria paroxistica nocturna infobioquimica. Natural history of paroxysmal nocturnal hemoglobinuria. The paroxysmal nocturnal hemoglobinuria is an infrequent cause of pancytopenia. Principais sintomas da hemoglobinuria paroxistica noturna e. Paroxysmal nocturnal hemoglobinuria pnh is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis. Paroxysmal nocturnal hemoglobinuria internal medicine usmle step 2.

The molecular basis of paroxysmal nocturnal hemoglobinuria. Dolores fraga fuentes, juan carlos valenzuela gamez. Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria. Arango elkin fernando, arroyave claudia lucia, zuleta john jairo, restrepo maria victoria, lopera antonio jose, zuluaga hector, botero juan pablo, zapata silvia. Paroxysmal nocturnal hemoglobinuria blood american. Scottish charles 1794 cirurgiao strubing paul 1882 urina escura matutina, hemolise intravascular, trombose. Hemoglobinuria paroxistica nocturna linkedin slideshare. 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. Hemoglobinuria paroxistica nocturna hematologia method of choice for diagnosis. Jan 02, 2019 paroxysmal nocturnal hemoglobinuria pnh was previously classified as purely an acquired hemolytic anemia due to a hematopoietic stem cell mutation defect.

Pnh results in the death of approximately 50% of affected individuals due to thrombotic complications and, until recently, had no specific therapy. Fisiopatologia e diagnostico da hemoglobinuria paroxistica. People with paroxysmal nocturnal hemoglobinuria have sudden, recurring episodes of symptoms paroxysmal symptoms, which may be triggered by stresses on the body, such as infections or physical. 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. 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. Hemoglobinuria paroxistica nocturna mananas oscuras, atardeceres. Marchiafava, 1911 michelli, 1931 anemia hemolitica com esplenomegalia e hemoglobinuria e hemosiderinuria ham th, dingle jh. This destructive process occurs due to the presence of defective surface protein daf on the red blood cell, which normally. Eculizumab and allogeneic bone marrow transplantation is the only effective therapies. Hemoglobinuria paroxistica nocturna by prezi user on. Hemoglobinuria paroxistica nocturna semantic scholar.

Hemoglobinuria paroxistica nocturna sistema complementario. Hemoglobinuria paroxistica noturna dos sintomas ao. It is a result of a somatic clonal mutation of one gene that is located in the. Oct 30, 2014 paroxysmal nocturnal hemoglobinuria pnh is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis. Mar, 2016 16 hemoglobinuria paroxistica nocturna dra diana otero. Paroxysmal nocturnal hemoglobinuria affects both sexes equally, and can occur at any age, although it is most often diagnosed in young adulthood. Natural history of paroxysmal nocturnal hemoglobinuriahillmen p, et al hospital. Existem tres caminhos pelos quais o complemento e ativado. Gaona c gaona, carlos almaguer hemoglobinuria paroxistica nocturna. Apresentase frequentemente com infeccoes recorrentes, neutropenia e trombocitopenia, e surge em associacao com outras doencas. Paroxysmal nocturnal hemoglobinuria genetics home reference.