Splanchnic vein thrombosis in myeloproliferative neoplasms: risk factors for recurrences in a cohort of 181 patients.
We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.
Institute of Hematology, Catholic University, Roma, Italy.
V
V
De Stefano
Center for Research and Innovation of Myeloproliferative Neoplasms, A.O.U. Careggi, University of Florence, Florence, Italy.
A M
AM
Vannucchi
Ospedale San Bortolo, Vicenza, Italy.
M
M
Ruggeri
Hospital Clínic IDIBAPS, Barcelona, Spain.
F
F
Cervantes
Department of Hematology, Hospital del Mar-IMIM, Barcelona, Spain.
A
A
Alvarez-Larrán
Oncohematology Division, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
A
A
Iurlo
Clinica Medica 1, Università di Padova, Padova, Italy.
M L
ML
Randi
Center for Research and Innovation of Myeloproliferative Neoplasms, A.O.U. Careggi, University of Florence, Florence, Italy.
L
L
Pieri
Institute of Hematology, Catholic University, Roma, Italy.
E
E
Rossi
Center for Research and Innovation of Myeloproliferative Neoplasms, A.O.U. Careggi, University of Florence, Florence, Italy.
P
0000-0003-1809-284X
P
Guglielmelli
Institute of Hematology, Catholic University, Roma, Italy.
S
S
Betti
Divisione di Ematologia, Ospedale San Gerardo, ASST Monza, Italy.
E
E
Elli
Hematology Division, ASST Papa Giovanni XXIII, Bergamo, Italy.
M C
MC
Finazzi
Hematology Division, ASST Papa Giovanni XXIII, Bergamo, Italy.
G
G
Finazzi
Skane University Hospital, Lund, Sweden.
E
E
Zetterberg
Institute of Hematology and Medical Oncology, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
N
N
Vianelli
Department of Translational Medicine, Università del Piemonte Orientale, Vercelli, Italy.
G
G
Gaidano
Ospedale San Bortolo, Vicenza, Italy.
I
I
Nichele
Oncohematology Division, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
D
D
Cattaneo
University Hospital of Olomouc, Olomouc, Czech Republic.
M
M
Palova
Department of Hematology, Institute Meir Medical Center, Kfar Saba, Israel.
M H
MH
Ellis
Haemostasis Unit, Department of Medical, Surgical and Advanced Technologies Sciences 'G.F. Ingrassia', University of Catania, Catania, Italy.
E
E
Cacciola
Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy.
A
A
Tieghi
Hospital Clinico, Valencia, Spain.
J C
JC
Hernandez-Boluda
A.O. Ospedale Niguarda Ca' Granda, Milano, Italy.
E
E
Pungolino
A.O. Universitaria, Policlinico di Bari, Italy.
G
G
Specchia
A.O. Santa Croce e Carle, Cuneo, Italy.
D
D
Rapezzi
IRCCS Ospedale San Raffaele, Milano, Italy.
A
A
Forcina
A.O. Universitaria, Messina, Italy.
C
C
Musolino
FROM Research Foundation, ASST Papa Giovanni XXIII, Bergamo, Italy.
A
A
Carobbio
Johannes Wesling Medical Center Minden, University of Bochum, Minden, Germany.
M
M
Griesshammer
FROM Research Foundation, ASST Papa Giovanni XXIII, Bergamo, Italy.
T
T
Barbui
D000328:Adult; D000368:Aged; D006502:Budd-Chiari Syndrome / Q000209:etiology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011087:Polycythemia Vera / Q000150:complications / Q000503:physiopathology*; D011169:Portal Vein / Q000503:physiopathology; D055728:Primary Myelofibrosis / Q000150:complications / Q000503:physiopathology*; D016016:Proportional Hazards Models; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D013920:Thrombocythemia, Essential / Q000150:complications / Q000503:physiopathology*; D020246:Venous Thrombosis / Q000209:etiology / Q000503:physiopathology*