Open AccessPF686 HEALTHCARE RESOURCE IMPLICATIONS IN PATIENTS WITH MYELOPROLIFERATIVE NEOPLASMS AND SPLANCHNIC VEIN THROMBOSIS
Author(s) -
Castellano X. Cabrera,
Craven B.,
Patch D.,
Yu D.,
Abiodun M.,
De Jesus J.,
Sekhar M.
Publication year - 2019
Publication title -
hemasphere
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 11
ISSN - 2572-9241
DOI - 10.1097/01.hs9.0000561028.61539.9c
Subject(s) - medicine , myeloproliferative neoplasm , portal vein thrombosis , polycythaemia , thrombosis , transjugular intrahepatic portosystemic shunt , radiology , thrombocytosis , gastroenterology , myelofibrosis , surgery , portal hypertension , bone marrow , cirrhosis , platelet
Background: 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. Aims: To assess the resource implications in MPN‐SVT patients at a tertiary London hospital. Methods: 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 algorithm 1 . 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. Results: 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.Summary/Conclusion: 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.