Results 31 to 40 of about 8,082 (209)

Paroxysmal Nocturnal Hemoglobinuria [PDF]

Blood, 2014
AbstractParoxysmal nocturnal hemoglobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias. The absence of two glycosylphosphatidylinositol (GPI)-anchored proteins, CD55 and CD59, leads to uncontrolled complement activation that accounts for hemolysis and other PNH ...
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A mutation in a functional Sp1 binding site of the telomerase RNA gene (hTERC) promoter in a patient with Paroxysmal Nocturnal Haemoglobinuria [PDF]

, 2004
Background<br/><br/> Mutations in the gene coding for the RNA component of telomerase, hTERC, have been found in autosomal dominant dyskeratosis congenita (DC) and aplastic anemia.
Ulku, B., Bessler, M., Bilsland, A., Mason, Philip J, Vulliamy, T., Serakinci, Nedime, Dokal, I., Ar, C., Ar, C, Marrone, Anna, Dokal, Inderjeet, Ulku, B, Bessler, Monica, Erzik, C, Serakinci, N, Serakinci, N., Marrone, A, Zhao, Jiangqin, Zhao, J, Dokal, I, Bilsland, A, Mason, PJ, Bilsland, Alan, Ulku, Birsen, Keith, W.N., Bessler, M, Vulliamy, T, Vulliamy, Tom, Keith, W Nicol, Erzik, Can, Marrone, A., Erzik, C., Zhao, J., Mason, P.J., Ar, Cem, Keith, WN   +35 more
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The kidneys in paroxysmal nocturnal hemoglobinuria [PDF]

Blood, 1981
Long-term study of 21 PNH patients revealed an unexpectedly high incidence of functional and anatomic renal abnormalities. Most patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. Evaluation of renal function revealed hyposthenuria, abnormal tubular function, and declining creatinine clearance ...
D A, Clark, S A, Butler, V, Braren, R C, Hartmann, D E, Jenkins   +4 more
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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 Nocturnal Hemoglobinuria

Current Opinion in Hematology, 2012
The aim is to report on recent observations related to the natural history of paroxysmal nocturnal hemoglobinuria (PNH) and to review new therapeutic strategies for controlling the hemolysis of PNH.This review focuses on studies designed to characterize the long-term outcome of patients with PNH treated with eculizumab and to define the relationship ...
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Frequency of Paroxysmal Nocturnal Hemoglobinuria Clone in Turkish Myelodysplastic Syndrome Group [PDF]

, 2018
Aim:Retrospective, cross-sectional, observational study to examine the frequency and features of paroxysmal nocturnal hemoglobinuria (PNH) clones in patients with myelodysplastic syndrome (MDS).Methods:Data were analyzed from the medical files of 41 MDS ...
Mesut Ayer, Tayfun Elibol, Merve Öztürk Çiloğlu, Fuat Şar, Esra Hayriye Ataoğlu, Fatma Aylin Ayer, Onur Hakkı Kırkızlar   +6 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 ...
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Paroxysmal Nocturnal Hemoglobinuria in Myelofibrosis [PDF]

Blood, 1970
Abstract Ten consecutive patients with myelofibrosis were examined for the following signs of PNH: Ham’s test, the sucrose hemolysis test, low red cell acetylcholinesterase (ACHE) activity and intravascular hemolysis. Two of the patients displayed all these signs and also had clinical symptoms of PNH.
N E, Hansen, S A, Killmann
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Clinical and molecular determinants of clonal evolution in aplastic anemia and paroxysmal nocturnal hemoglobinuria [PDF]

, 2023
Secondary myeloid neoplasms (sMNs) remain the most serious long-term complications in patients with aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH).
Catto, Luiz Fernando B, Hernandez, Lucie, Socié, Gérard, Allain, Vincent, Prata, Pedro, Calado, Rodrigo T, Peffault de Latour, Régis, Inacio, Mariana C B, Pinto, Andre L, Maciejewski, Jaroslaw P, Calado, Rodrigo, Sicre de Fontbrune, Flore, Visconte, Valeria, Inacio, Mariana C.B., Caillat-Zucman, Sophie, Voso, Maria Teresa, Patel, Bhumika J, Larcher, Lise, Galimard, Jacques-Emmanuel, Durmaz, Arda, Clappier, Emmanuelle, Inacio, Mariana, Voso, Maria, Patel, Bhumika, Gurnari, Carmelo, Prata, Pedro Henrique, Maciejewski, Jaroslaw, Soulier, Jean, Pagliuca, Simona, Catto, Luiz, Catto, Luiz Fernando B., Dhedin, Nathalie, Sebert, Marie, Pinto, Andre   +33 more
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Model-Informed Precision Dosing of Eculizumab in Patients with Paroxysmal Nocturnal Hemoglobinuria. [PDF]

Clin Pharmacokinet
Contains fulltext : 322381.pdf (Publisher’s version ) (Open Access)BACKGROUND AND OBJECTIVE: Eculizumab is an expensive therapeutic monoclonal antibody inhibiting complement C5 and approved for various indications, including the rare ...
Ter Avest M, Langemeijer SMC, van den Heuvel LPWJ, Baas LM, van de Kar NCAJ, Ter Heine R.   +5 more
europepmc   +2 more sources