Frontiers in Oncology (Mar 2023)
Thromboembolic events in patients with paroxysmal nocturnal hemoglobinuria (PNH): Real world data of a Greek nationwide multicenter retrospective study
- S. Chatzileontiadou,
- E. Hatjiharissi,
- M. Angelopoulou,
- J. V. Asimakopoulos,
- N. E. Loutsidi,
- T. Chatzikonstantinou,
- P. Zikos,
- A. Bouchla,
- Z. Bezirgiannidou,
- E. Kouvata,
- C. Frouzaki,
- P. Chaloudis,
- D. Sotiropoulos,
- V. Douka,
- A. Sirigou,
- E. Mandala,
- M. Psyllaki,
- H. A. Papadaki,
- T. Marinakis,
- N. A. Viniou,
- S. Kokkori,
- F. Kontopidou,
- A. Skepetari,
- G. Vassilopoulos,
- I. Kotsianidis,
- V. Pappa,
- C. Lalayanni,
- I. Baltadakis,
- S. Delimpassi,
- M. Pagoni,
- M. Papaioannou
Affiliations
- S. Chatzileontiadou
- Hematology Unit, 1st Department of Internal Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
- E. Hatjiharissi
- Hematology Unit, 1st Department of Internal Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
- M. Angelopoulou
- Department of Hematology, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
- J. V. Asimakopoulos
- Department of Hematology, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
- N. E. Loutsidi
- Hematology - Lymphomas Department and Bone Marrow Transplant Unit, Evangelismos General Hospital, Athens, Greece
- T. Chatzikonstantinou
- Department of Hematology, Bone Marrow Transplantation Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
- P. Zikos
- Department of Hematology, General Hospital of Patras “Aghios Andreas”, Patras, Greece
- A. Bouchla
- Second Department of Internal Medicine and Research Unit, Hematology Unit, University General Hospital, “Attikon”, Athens, Greece
- Z. Bezirgiannidou
- Department of Hematology, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece
- E. Kouvata
- Department of Hematology, University Hospital of Larissa, Larissa, Greece
- C. Frouzaki
- Hematology Unit, 1st Department of Internal Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
- P. Chaloudis
- Hematology Unit, 1st Department of Internal Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
- D. Sotiropoulos
- Department of Hematology, Bone Marrow Transplantation Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
- V. Douka
- Department of Hematology, Bone Marrow Transplantation Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
- A. Sirigou
- Department of Hematology, Bone Marrow Transplantation Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
- E. Mandala
- Forth Department of Medicine, Aristotle University of Thessaloniki, “Hippokration” Hospital, Thessaloniki, Greece
- M. Psyllaki
- 0Department of Hematology, University of Crete School of Medicine, University Hospital of Heraklion, Heraklion, Greece
- H. A. Papadaki
- 0Department of Hematology, University of Crete School of Medicine, University Hospital of Heraklion, Heraklion, Greece
- T. Marinakis
- 1Department of Hematology, “G. Gennimatas” General Hospital, Athens, Greece
- N. A. Viniou
- 2Hematology Unit, First Department of Internal Medicine, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece
- S. Kokkori
- 3Hematology Laboratory, University General Hospital “Attikon”, Athens, Greece
- F. Kontopidou
- 42nd Department of Internal Medicine, Medical School, University of Athens, Hippokration General Hospital, Athens, Greece
- A. Skepetari
- 42nd Department of Internal Medicine, Medical School, University of Athens, Hippokration General Hospital, Athens, Greece
- G. Vassilopoulos
- Department of Hematology, University Hospital of Larissa, Larissa, Greece
- I. Kotsianidis
- Department of Hematology, University Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece
- V. Pappa
- Second Department of Internal Medicine and Research Unit, Hematology Unit, University General Hospital, “Attikon”, Athens, Greece
- C. Lalayanni
- Department of Hematology, Bone Marrow Transplantation Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
- I. Baltadakis
- Hematology - Lymphomas Department and Bone Marrow Transplant Unit, Evangelismos General Hospital, Athens, Greece
- S. Delimpassi
- Hematology - Lymphomas Department and Bone Marrow Transplant Unit, Evangelismos General Hospital, Athens, Greece
- M. Pagoni
- Hematology - Lymphomas Department and Bone Marrow Transplant Unit, Evangelismos General Hospital, Athens, Greece
- M. Papaioannou
- Hematology Unit, 1st Department of Internal Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
- DOI
- https://doi.org/10.3389/fonc.2023.1128994
- Journal volume & issue
-
Vol. 13
Abstract
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.
Keywords
- paroxysmal nocturnal hemoglobinuria (PNH)
- thrombosis
- hemolytic anemia
- orphan disease
- complement inhibitors
