Results 11 to 20 of about 7,137 (182)

Paroxysmal nocturnal hemoglobinuria and telomere length predicts response to immunosuppressive therapy in pediatric aplastic anemia

Haematologica, 2015
Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length
Atsushi Narita, Hideki Muramatsu, Yuko Sekiya, Yusuke Okuno, Hirotoshi Sakaguchi, Nobuhiro Nishio, Nao Yoshida, Xinan Wang, Yinyan Xu, Nozomu Kawashima, Sayoko Doisaki, Asahito Hama, Yoshiyuki Takahashi, Kazuko Kudo, Hiroshi Moritake, Masao Kobayashi, Ryoji Kobayashi, Etsuro Ito, Hiromasa Yabe, Shouichi Ohga, Akira Ohara, Seiji Kojima   +21 more
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Paroxysmal Nocturnal Hemoglobinuria [PDF]

Blood, 1950
Abstract 1. Paroxysmal noctural hemoglobinuria is believed to be an acquired disease of the hematopoietic system in which abnormal red cells, white cells, and platelets are produced. The lesion of the cells probably involves the stromal proteins in such a fashion that they are susceptible to the proteolytic effect of a sytem of normal ...
openaire   +2 more sources

Detection of paroxysmal nocturnal hemoglobinuria clones in patients with myelodysplastic syndromes and related bone marrow diseases, with emphasis on diagnostic pitfalls and caveats

Haematologica, 2009
Background The presence of paroxysmal nocturnal hemoglobinuria clones in the setting of aplastic anemia or myelodysplastic syndrome has been shown to have prognostic and therapeutic implications. However, the status of paroxysmal nocturnal hemoglobinuria
Sa A. Wang, Olga Pozdnyakova, Jeffrey L. Jorgensen, L. Jeffrey Medeiros, Dariusz Stachurski, Mary Anderson, Azra Raza, Bruce A. Woda   +7 more
doaj   +1 more source

PAROXYSMAL HEMOGLOBINURIA [PDF]

Archives of Internal Medicine, 1910
In the case presented in this article, on which our work was performed, any severe chilling of the patient's skin caused a temporary hemoglobinuria, drowsiness, regurgitation of food and other minor symptoms to be mentioned later. The phenomena were produced either by a chilling of the general body surface or, what was more common, by the mere exposure
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Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil

Revista Brasileira de Hematologia e Hemoterapia, 2011
BACKGROUND: Paroxysmal nocturnal hemoglobinuria is a hematological disease with complex physiopathology. It is genetically characterized by a somatic mutation in the PIG-A gene (phosphatidylinositol glycan anchor biosynthesis, class A), in which the best
Lacy Cardoso de Brito Junior, Maria do Socorro de Oliveira Cardoso, Euzamar Gaby Rocha, Herika Anijar, Mariana Cunha, João Carlos Pina Saraiva   +5 more
doaj   +1 more source

Paroxysmal nocturnal hemoglobinuria clone in 103 Brazilian patients: diagnosis and classification

Revista Brasileira de Hematologia e Hemoterapia, 2015
Background: Paroxysmal nocturnal hemoglobinuria is an acquired chronic hemolytic ane- mia, which often manifests as peripheral blood cytopenias and thrombosis. Objective: The aim of this study is to describe a Brazilian population of paroxysmal nocturnal
Ana Paula de Azambuja, Mariester Malvezzi, Marco Antonio Bitencourt, Michel Michels Oliveira, Larissa Alessandra Medeiros, Ricardo Pasquini   +5 more
doaj   +1 more source

Loss of expression of neutrophil proteinase-3: a factor contributing to thrombotic risk in paroxysmal nocturnal hemoglobinuria

Haematologica, 2011
Background A deficiency of specific glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria may be responsible for most of the clinical features of this disease, but some functional consequences may be indirect. For example,
Anna M. Jankowska, Hadrian Szpurka, Mark Calabro, Sanjay Mohan, Andrew E. Schade, Michael Clemente, Roy L. Silverstein, Jaroslaw P. Maciejewski   +7 more
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Danazol for paroxysmal nocturnal hemoglobinuria [PDF]

American Journal of Hematology, 1997
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal stem-cell disorder in which blood cells lack complement inhibiting membrane proteins, and become susceptible to complement-mediated injury, leading to chronic intravascular hemolysis and pancytopenia. Glucocorticoids have been a mainstay of therapy.
W J, Harrington, L, Kolodny, L L, Horstman, W, Jy, Y S, Ahn   +4 more
openaire   +2 more sources

Small-molecule factor D inhibitors selectively block the alternative pathway of complement in paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome

Haematologica, 2017
Paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome are diseases of excess activation of the alternative pathway of complement that are treated with eculizumab, a humanized monoclonal antibody against the terminal complement ...
Xuan Yuan, Eleni Gavriilaki, Jane A. Thanassi, Guangwei Yang, Andrea C. Baines, Steven D. Podos, Yongqing Huang, Mingjun Huang, Robert A. Brodsky   +8 more
doaj   +1 more source

Paroxysmal nocturnal hemoglobinuria in systemic lupus erythematosus: a case report

Journal of Medical Case Reports, 2011
Introduction Paroxysmal nocturnal hemoglobinuria is an acquired disorder of hemopoiesis and is characterized by recurrent episodes of intravascular hemolysis due to an increased sensitivity to complement-mediated hemolysis.
Nakamura Norio, Sugawara Toshiyuki, Shirato Ken-ichi, Kumasaka Ryuichiro, Nakamura Masayuki, Shimada Michiko, Fujita Takeshi, Murakami Reiichi, Shimaya Yuko, Osawa Hiroshi, Yamabe Hideaki, Okumura Ken   +11 more
doaj   +1 more source