Results 21 to 30 of about 8,824 (214)

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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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]

Journal of the American Medical Association, 1914
History. —J., a man aged 45, Swedish, a sheet-metal worker, entered the Peter Bent Brigham Hospital, Sept. 11, 1913, complaining of chills, fever and bloody urine. The family history was unimportant as related to the present illness. The patient had interstitial keratitis at 3 years (?), and measles and small-pox as a child, but no other infectious ...
openaire   +2 more sources

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

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

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
openaire   +2 more sources

Iptacopan monotherapy resulted in increased hemoglobin level in patients with PNH and hemoglobin ≥10 g/dL after anti-C5 therapy. [PDF]

Hemasphere
Abstract Patients with paroxysmal nocturnal hemoglobinuria (PNH) on anti‐C5 often experience extravascular hemolysis with anemia. Iptacopan, the first oral proximal complement inhibitor targeting factor B, has shown efficacy and safety in PNH patients. APPULSE‐PNH (NCT05630001), a phase 3b, single‑arm, open‐label trial, enrolled adult patients with PNH
Kulasekararaj A, de Fontbrune FS, Gaya A, Weitz I, Kuter DJ, Patel BJ, Araten DJ, Singh A, Jang JH, Kelly RJ, Di Bona E, Loschi M, Pullarkat V, Schubert J, Notaro R, Yenerel MN, Beckman J, Blin N, Murakhovskaya I, Panse J, Roman E, Röth A, Schrezenmeier H, Tantravahi S, de Latour RP, Mahajan N, Monaco L, Ding T, Lawniczek T, Ferber P, Dahlke M, Risitano AM.   +31 more
europepmc   +2 more sources

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 ...
openaire   +6 more sources

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
core   +1 more source

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
core   +1 more source