Thromboembolic events in patients with paroxysmal nocturnal hemoglobinuria (PNH): Real world data of a Greek nationwide multicenter retrospective study

被引:11
作者
Chatzileontiadou, S. [1 ]
Hatjiharissi, E. [1 ]
Angelopoulou, M. [2 ]
Asimakopoulos, J. V. [2 ]
Loutsidi, N. E. [3 ]
Chatzikonstantinou, T. [4 ]
Zikos, P. [5 ]
Bouchla, A. [6 ,7 ]
Bezirgiannidou, Z. [8 ]
Kouvata, E. [9 ]
Frouzaki, C. [1 ]
Chaloudis, P. [1 ]
Sotiropoulos, D. [4 ]
Douka, V. [4 ]
Sirigou, A. [4 ]
Mandala, E. [10 ]
Psyllaki, M. [11 ]
Papadaki, H. A. [11 ]
Marinakis, T.
Viniou, N. A.
Kokkori, S.
Kontopidou, F.
Skepetari, A.
Vassilopoulos, G. [9 ]
Kotsianidis, I. [8 ]
Pappa, V. [6 ,7 ]
Lalayanni, C. [4 ]
Baltadakis, I. [3 ]
Delimpassi, S. [3 ]
Pagoni, M. [3 ]
Papaioannou, M. [1 ]
机构
[1] Aristotle Univ Thessaloniki, AHEPA Univ Hosp, Dept Internal Med 1, Hematol Unit, Thessaloniki, Greece
[2] Natl & Kapodistrian Univ Athens, Laikon Gen Hosp, Dept Hematol, Athens, Greece
[3] Evangelismos Gen Hosp, Hematol Lymphomas Dept, Bone Marrow Transplant Unit, Athens, Greece
[4] G Papanicolaou Hosp, Dept Hematol, Bone Marrow Transplantat Unit, Thessaloniki, Greece
[5] Gen Hosp Patras Aghios Andreas, Dept Hematol, Patras, Greece
[6] Univ Gen Hosp, Dept Internal Med 2, Athens, Greece
[7] Univ Gen Hosp, Res Unit, Hematol Unit, Athens, Greece
[8] Democritus Univ Thrace, Univ Hosp Alexandroupolis, Dept Hematol, Alexandroupolis, Greece
[9] Univ Hosp Larissa, Dept Hematol, Larisa, Greece
[10] Aristotle Univ Thessaloniki, Hippokrat Hosp, Dept Med 4, Thessaloniki, Greece
[11] Univ Crete, Univ Hosp Heraklion, Sch Med, Dept Hematol, Iraklion, Greece
来源
FRONTIERS IN ONCOLOGY | 2023年 / 13卷
关键词
paroxysmal nocturnal hemoglobinuria (PNH); thrombosis; hemolytic anemia; orphan disease; complement inhibitors; STEM-CELL TRANSPLANTATION; NATURAL-HISTORY; ECULIZUMAB; THROMBOSIS; COMPLICATIONS; MANAGEMENT;
D O I
10.3389/fonc.2023.1128994
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Thrombosis is the most common and a life-threatening complication in patients with Paroxysmal Nocturnal Hemoglobinuria. One-third of patients with PNH experience at least one thromboembolic event during the course of the disease, with thrombosis being the most common cause of death in these patients. The mechanism of thrombosis in PNH is complex and continues to be of great research interest. Since the introduction of C5 complement inhibitors in the treatment of PNH, the incidence of thromboembolic events has decreased substantially. We retrospectively analyzed data concerning the thrombotic episodes of 41 patients with PNH from 14 different national hematology centers in Greece. Sixteen patients (39%) experienced at least one episode of thrombosis, including, seven (43.8%) at diagnosis, seven (43.8%) during the course of the disease and two (12.5%) patients prior to PNH diagnosis. Nearly half of these individuals (n=7, 43.8%) had multiple episodes of thrombosis during the course of their disease. The most common sites of thrombosis were intra-abdominal veins. Three out of 26 patients developed thrombosis while on eculizumab. In none of the 16 patients, the thrombotic event was fatal. Our findings, despite the small number of patients, confirmed that thrombosis continues to be a significant complication of PNH affecting more than one third of the patients.
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页数:8
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共 38 条
  • [1] Paroxysmal nocturnal hemoglobinuria: current treatments and unmet needs
    Bektas, Meryem
    Copley-Merriman, Catherine
    Khan, Shahnaz
    Sarda, Sujata P.
    Shammo, Jamile M.
    [J]. JOURNAL OF MANAGED CARE & SPECIALTY PHARMACY, 2020, 26 (12) : S14 - S20
  • [2] How I treat paroxysmal nocturnal hemoglobinuria
    Brodsky, Robert A.
    [J]. BLOOD, 2021, 137 (10) : 1304 - 1309
  • [3] Paroxysmal nocturnal hemoglobinuria
    Brodsky, Robert A.
    [J]. BLOOD, 2014, 124 (18) : 2804 - 2811
  • [4] JAK2V617F positive polycythemia vera with paroxysmal nocturnal hemoglobinuria and visceral thromboses: a case report and review of the literature
    Chatzidavid, Sevastianos
    Giannakopoulou, Nefeli
    Diamantopoulos, Panagiotis Theodorou
    Gavriilaki, Eleni
    Katsiampoura, Panagiota
    Lakiotaki, Eleftheria
    Sakellariou, Stratigoula
    Viniou, Nora-Athina
    Dryllis, Georgios
    [J]. THROMBOSIS JOURNAL, 2021, 19 (01)
  • [5] Paroxysmal nocturnal hemoglobinuria:: natural history of disease subcategories
    de Latour, Regis Peffault
    Mary, Jean Yves
    Salanoubat, Celia
    Terriou, Louis
    Etienne, Gabriel
    Mohty, Mohamad
    Roth, Sophie
    de Guibert, Sophie
    Maury, Sebastien
    Cahn, Jean Yves
    Socie, Gerard
    [J]. BLOOD, 2008, 112 (08) : 3099 - 3106
  • [6] Successful discontinuation of anticoagulation following eculizumab administration in paroxysmal nocturnal hemoglobinuria
    Emadi, Ashkan
    Brodsky, Robert A.
    [J]. AMERICAN JOURNAL OF HEMATOLOGY, 2009, 84 (10) : 699 - 701
  • [7] Development of paroxysmal nocturnal hemoglobinuria in CALR-positive myeloproliferative neoplasm
    Fraiman, Yarden S.
    Cuka, Nathan
    Batista, Denise
    Vuica-Ross, Milena
    Moliterno, Alison R.
    [J]. JOURNAL OF BLOOD MEDICINE, 2016, 7 : 107 - 110
  • [8] Allogeneic hematopoietic stem cell transplantation for paroxysmal nocturnal hemoglobinuria: a retrospective single-center study
    Fumani, Hosein Kamranzadeh
    Zokaasadi, Mohammad
    Kasaeian, Amir
    Alimoghaddam, Kamran
    Mousavi, Seyed Asadollah
    Bahar, Babak
    Vaezi, Mohammad
    Ghavamzadeh, Ardeshir
    [J]. HEMATOLOGICAL ONCOLOGY, 2017, 35 (04) : 935 - 938
  • [9] Gaidano V., 2017, HEMATOL MED ONCOL, V2, P1, DOI [10.15761/HMO.1000132, DOI 10.15761/HMO.1000132]
  • [10] A 15-year, single institution experience of anticoagulation management in paroxysmal nocturnal hemoglobinuria patients on terminal complement inhibition with history of thromboembolism
    Gerber, Gloria F.
    DeZern, Amy E.
    Chaturvedi, Shruti
    Brodsky, Robert A.
    [J]. AMERICAN JOURNAL OF HEMATOLOGY, 2022, 97 (02) : E59 - E62