Nnhemoglobinuria paroxistica nocturna pdf 2011

Tratamiento hemoglobinuria paroxistica nocturna medicinatv. Paroxysmal nocturnal hemoglobinuria pnh is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis. 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. Fisiopatologia e diagnostico da hemoglobinuria paroxistica. Hemoglobinuria paroxistica noturna diagnostico e tratamento dra. Paroxysmal nocturnal hemoglobinuria is a hematological disease with complex physiopathology. We report the case of a 32 yearold female pnh patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with pnh, evolving in three years with buddchiari syndrome and liver transplantation. Bradfor teaching hospitals, nhs foundation trust and university y hospital st. Apresentase frequentemente com infeccoes recorrentes, neutropenia e trombocitopenia, e surge em associacao com outras doencas. Lima, in 2011, as guidance, ensued with detailed reading of selected articles.

Marchiafava, 1911 michelli, 1931 anemia hemolitica com esplenomegalia e hemoglobinuria e hemosiderinuria ham th, dingle jh. Paroxysmal nocturnal hemoglobinuria blood american. Pnh results in the death of approximately 50% of affected individuals due to thrombotic complications and, until recently, had no specific therapy. Hemoglobinuria paroxistica noturna causas e tratamentos.

It is genetically characterized by a somatic mutation in the piga gene phosphatidylinositol glycan anchor biosynthesis, class a, in which the best known antigens are daf decay accelerating factor or cd55 and mirl membrane inhibitor of reactive lysis or cd59. Hemoglobinuria paroxistica nocturna linkedin slideshare. Paroxysmal nocturnal hemoglobinuria pnh is a clonal disorder of the hematopoietic stem cell hartmann and arnold 1977 resulting in the production of blood cells which are defective in that they. Jan 02, 2019 paroxysmal nocturnal hemoglobinuria pnh was previously classified as purely an acquired hemolytic anemia due to a hematopoietic stem cell mutation defect. Sep 05, 2017 hemoglobinuria paroxistica nocturna 1. 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. 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.

Early differentiation of the type or cause of discolored urine is necessary for accurate diagnosis and prompt management. Hemoglobinuria paroxistica noturna diagnostico e tratamento. Scottish charles 1794 cirurgiao strubing paul 1882 urina escura matutina, hemolise intravascular, trombose. Nocturna hematologia method of choice for diagnosis. Report of a case of paroxysmal nocturnal hemoglobinuria pnh. Discolored urine is a common reason for office visits to a primary care physician and urology referral. Often, theres renal or lung involvement, with morbimortality predominantly linked to thromboembolic events. Frequency of paroxysmal nocturnal hemoglobinuria in patients. The paroxysmal nocturnal hemoglobinuria pnh is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. Eculizumab and allogeneic bone marrow transplantation is the only effective therapies. Sintomas hemoglobinuria paroxistica nocturna medicinatv. Agencia espanola del medicamento y productos sanitarios. Pnh is a rare acquired disease, presenting as hemolysis classic pnh or aplastic anemia.

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 reactions. Nydia strachman bacal hematologista e patologista clinica mba em economia e gestao na saude. Hemoglobinuria paroxistica nocturna cesar david ordonez dominguez 2. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Dolores fraga fuentes, juan carlos valenzuela gamez.