Poster Session I: Myeloproliferative neoplasms - Clinical
Splanchnic vein thrombosis (SVT) is strongly associated with Myeloproliferative neoplasms (MPN) and isolated JAK2v617f mutation. MPN-SVT is associated with significant disease-related and iatrogenic complications. Patients' service needs are therefore complex.
To assess the resource implications in MPN-SVT patients at a tertiary London hospital.
MPN-SVT was defined as thrombosis of the portal (PVT), hepatic (BCS), superior mesenteric (SMVT), splenic (SpT) vein singly or in combination plus a diagnosis of MPN including: Polycythaemia Vera (PV), Essential Thrombocythaemia (ET), Myelofibrosis (MF), MPN unclassified (MPNu), Myelodysplastic syndrome-MPN (MDS-MPN) or isolated JAK2v617f mutation. 67 patients were identified from an existing database of patients, 16 excluded due to incomplete records. 51 patients were followed for a median of 5 years (range 1-25). Data was collected through the IT systems at our hospital and shared cared hospitals. Patients were treated to standard algorithm1. Resources analysed: imaging (CT, MRI, US), procedures (endoscopy, bone marrow (BM) biopsy, interventions) and treatment (anticoagulation, aspirin, cytoreductive agents). Clinical outcomes and overall QOL were also recorded.
52% were female, 48% male. Median age at SVT diagnosis: 42 (range 16-71). 16 had PV, 12 MF, 11 ET, 7 isolated JAK2v617f, 5 other. SVT at presentation: 41% isolated PVT, 27% BCS, 26% combined PVT and 6% isolated SpT. Median number of scans related to diagnosis: 8 (range 1-39) (Table 1). Median of 4 CT scans (0-19), 4 US (0-21) and 1 (0-18) MRI. 19 patients underwent Transjugular intrahepatic portosystemic shunt (TIPSS) −2 were unsuccessful- 15/17 had TIPSograms, median of 4 TIPSograms/patient. 35/51 patients had endoscopy with a median of 2. 43/51 patients had a BM biopsy. 2 patients had liver transplant and 1 is awaiting intestinal and liver transplant. 7/51 underwent surgery needing ITU care. 48/51 received warfarin, 2 received DOACs and 1 received no anticoagulation (long-standing stable cavernoma). 23 patients received additional aspirin (13 with TIPSS). 82% received cytoreduction: 31 hydroxycarbamide, 19 ruxolitinib, 6 interferon; 10 had venesections. 29% had a new thrombosis, mainly venous (80%). Of these, 47% were new SVTs (4/7 with prior TIPSS, 1/7 prior thrombolysis). 17/51 (33%) had a new bleed, one fatal; 8 were on warfarin + aspirin. 20% had both a new thrombosis and a new bleed. 4 patients died, causes of death were: splenic aneurysm rupture, liver failure, leukemic transformation and infection. 29 patients completed at least one Symptom Assessment Form (SAF). Median overall QOL score of 4 (0 = best, 10 = worst). Median score 8 in PV and 2 in isolated JAK2v617f.
MPN-SVT is rare but resource-intensive with high levels of morbidity and a significant effect on patients' QOL. Our patients were managed intensively, despite this, fatality was 8% and recurrence of thrombosis 29%. These data highlight the urgent need to develop care pathways, treatment algorithms and establish clinical trials.