NISHIMURA, Jun-ichi, Kiyoshi ANDO, Masayoshi MASUKO, Hideyoshi NOJI, Yoshikazu ITO, Jiří MAYER, Laimonas GRISKEVICIUS, Christoph BUCHER, Florian MULLERSHAUSEN, Peter GERGELY, Izabela ROZENBERG, Anna SCHUBART, Raghav CHAWLA, Jean-Michel RONDEAU, Michael ROGUSKA, Igor SPLAWSKI, Mark T KEATING, Leslie JOHNSON, Rambabu DANEKULA, Morten BAGGER, Yoko WATANABE, Borje HARALDSSON and Yuzuru KANAKURA. Tesidolumab (LFG316) for treatment of C5-variant patients with paroxysmal nocturnal hemoglobinuria. Haematologica. PAVIA: FERRATA STORTI FOUNDATION, 2022, vol. 107, No 6, p. 1483-1488. ISSN 0390-6078. Available from: https://dx.doi.org/10.3324/haematol.2020.265868.
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Basic information
Original name Tesidolumab (LFG316) for treatment of C5-variant patients with paroxysmal nocturnal hemoglobinuria
Authors NISHIMURA, Jun-ichi (guarantor), Kiyoshi ANDO, Masayoshi MASUKO, Hideyoshi NOJI, Yoshikazu ITO, Jiří MAYER (203 Czech Republic, belonging to the institution), Laimonas GRISKEVICIUS, Christoph BUCHER, Florian MULLERSHAUSEN, Peter GERGELY, Izabela ROZENBERG, Anna SCHUBART, Raghav CHAWLA, Jean-Michel RONDEAU, Michael ROGUSKA, Igor SPLAWSKI, Mark T KEATING, Leslie JOHNSON, Rambabu DANEKULA, Morten BAGGER, Yoko WATANABE, Borje HARALDSSON and Yuzuru KANAKURA.
Edition Haematologica, PAVIA, FERRATA STORTI FOUNDATION, 2022, 0390-6078.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30205 Hematology
Country of publisher Italy
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 10.100
RIV identification code RIV/00216224:14110/22:00128073
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.3324/haematol.2020.265868
UT WoS 000836816100029
Keywords in English Tesidolumab; paroxysmal nocturnal hemoglobinuria; C5-variant patients
Tags 14110212, rivok
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 18/1/2023 09:59.
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is caused by clonal expansion of hematopoietic stem cells that carry a somatic mutation in the X-linked gene PIG-A (phosphatidylinositol glycan anchor biosynthesis class A). The mutation leads to a deficiency of glycosylphosphatidylinositol-anchored membrane proteins. The loss of membrane-associated complement regulatory proteins CD55 and CD59 increases susceptibility of red blood cells and platelets to complement-mediated lysis, leading to hemolytic anemia, thrombophilia and reduced life expectancy in untreated patients.
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