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Paroxysmal nocturnal hemoglobinuria: from physiopathology to treatment. Unitermos: Resultado de tratamento. Paroxysmal nocturnal hemoglobinuria PNH is a rare disorder, an acquired chronic hemolytic anemia, often associated with recurrent nocturnal exacerbations, recurrent infections, neutropenia, thrombocytopenia, and episodes of venous thrombosis. Its clinical course is highly variable.

It frequently arises in association with bone marrow failure, particularly aplastic anemia and myelodysplastic syndrome. It is also an acquired thrombophilia, presenting with a variety of venous thrombosis, mainly manifested with intra-abdominal thrombosis, here the major cause of mortality.

The triad of hemolytic anemia, pancytopenia, and thrombosis makes a truly unique clinical syndrome of PNH, which was reclassified from a purely acquired hemolytic anemia to a hematopoietic stem cell mutation defect of the phosphatidyl inositol glycanclass-A gene. This mutation results in an early block in the synthesis of glycosylphosphatidylinositol GPI anchors, responsible for binding membrane functional proteins. Among these proteins are the complement inhibitors, especially CD55 and CD59, that play a key role in protecting blood cells from complement cascade attack.

Therefore, in PNH occurs an increased susceptibility of red cells to complement, and consequently, hemolysis. We here review PNH physiopathology, clinical course, and treatment options, especially eculizumab, a humanized monoclonal antibody that blocks the activation of terminal complement at C5 and prevents formation of the terminal complement complex, the first effective drug therapy for PNH. Key words: Paroxysmal nocturnal hemoglobinuria.

Treatment outcome. Clinical symptoms. Estudos in vitro sugerem ainda que o complemento terminal possa ativar diretamente as plaquetas de pacientes com HPN 2. Em ele foi expandido e atualizado, somando pacientes, com mediana do tempo de acompanhamento de sete anos Eculizumab foi administrado por 52 semanas utilizando-se as doses descritas acima.

Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Impact of magnetic resonance imaging on the diagnosis of abdominal complications of paroxysmal nocturnal hemoglobinuria. Paroxysmal nocturnal hemoglobinuria with onset in childhood and adolescence. N Engl J Med. Besa EC. Paroxysmal nocturnal hemoglobinuria, in eMedicine. Crosby WH. Paroxysmal nocturnal hemoglobinuria; a classic description by Paul Strubling in , and a bibliography of the disease.

Parker CJ. Paroxysmal nocturnal hemoglobinuria: an historical overview. Paroxysmal nocturnal hemoglobinuria: relation of the clinical manifestations to underlying pathogenic mechanisms. Studies on destruction of red blood cells. J Clin Invest. Socie G, et al. Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology.

The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. Bessler M, Hiken J. The pathophysiology of disease in patients with paroxysmal nocturnal hemoglobinuria. Brodsky RA. Paroxysmal nocturnal hemoglobinuria: stem cells and clonality. Advances in the diagnosis and therapy of paroxysmal nocturnal hemoglobinuria.

Blood Rev. Br J Haematol. Smith LJ. Paroxysmal nocturnal hemoglobinuria. Clin Lab Sci. Walport MJ. First of two parts. Dacie J. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria.

Meletis J, Terpos E. Recent insights into the pathophysiology of paroxysmal nocturnal hemoglobinuria. Med Sci Monit. Rosse WF. Pathogenesis of paroxysmal nocturnal hemoglobinuria: missing cell proteins. UpToDate Bone marrow failure syndromes: paroxysmal nocturnal hemoglobinuria. Hematol Oncol Clin North Am. Aplastic anemia. Curr Opin Hematol. Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories. Clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria-type cells in bone marrow failure syndrome.

The "sugar-water" test for paroxysmal nocturnal hemoglobinuria. How I treat paroxysmal nocturnal hemoglobinuria. Immune lysis of normal human and paroxysmal nocturnal hemoglobinuria PNH red blood cells. The sensitivity of PNH red cells to lysis by complement and specific antibody.

The use of monoclonal antibodies and flow cytometry in the diagnosis of paroxysmal nocturnal hemoglobinuria. Neutrophil life span in paroxysmal nocturnal hemoglobinuria. Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan. Medicine Baltimore. Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria PNH. Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria.

Rosse W. Hematology: basic principals and practice. A blue kidney--chronic renal failure as a consequence of siderosis in paroxysmal nocturnal hemoglobinuria? Clin Nephrol. Sustained response and long-term safety of eculizumab in paroxysmal nocturnal hemoglobinuria. Detection of somatic mutations of the PIG-A gene in Brazilian patients with paroxysmal nocturnal hemoglobinuria. Braz J Med Biol Res. Bidoia BG. Role of phosphatidylinositol-linked proteins in paroxysmal nocturnal hemoglobinuria pathogenesis.

Annu Rev Med. Somatic mutations of the PIG-A gene found in Japanese patients with paroxysmal nocturnal hemoglobinuria. High incidence of thrombosis in African-American and Latin-American patients with paroxysmal nocturnal haemoglobinuria.

Thromb Haemost. Natural history of paroxysmal nocturnal haemoglobinuria using modern diagnostic assays. Paroxysmal nocturnal haemoglobinuria: variation in clinical severity and association with bone-marrow hypoplasia.

The aplastic anaemia--paroxysmal nocturnal haemoglobinuria syndrome. Nippon Ketsueki Gakkai Zasshi. Detection of paroxysmal nocturnal hemoglobinuria clones in patients with myelodysplastic syndromes and related bone marrow diseases, with emphasis on diagnostic pitfalls and caveats.

Natural history of paroxysmal nocturnal hemoglobinuria. Dacie JL. Paroxysmal nocturnal hemoglobinuria: clinical manifestation, haematology, and nature of the disease. Series Haematol. Paroxysmal nocturnal hemoglobinuria: clinical and laboratory studies relating to iron metabolism and therapy with androgen and iron.

Treatment of paroxysmal nocturnal hemoglobinuria. Bone marrow transplants for paroxysmal nocturnal haemoglobinuria. Bone marrow transplantation for paroxysmal nocturnal hemoglobinuria. Hematopoietic cell transplantation from related and unrelated donors after minimal conditioning as a curative treatment modality for severe paroxysmal nocturnal hemoglobinuria.

Biol Blood Marrow Transplant.

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