This handout is for dvt in jak2-v617f-positive myeloproliferative neoplasm (pv / et / pmf). Your care team identified this based on: unilateral leg swelling in patient with aquagenic pruritus, erythromelalgia, splenomegaly, or unexplained polycythemia / thrombocytosis — suspect underlying mpn.
Other reasons your team may use this plan: splanchnic vein thrombosis (budd-chiari, portal, mesenteric, splenic) or cerebral venous sinus thrombosis as index event — jak2-v617f testing mandatory even with normal cbc; cbc at presentation with hb > 16.5 (men) / > 16.0 (women), platelets > 450 × 10⁹/l, or wbc > 11 — order jak2-v617f pcr; recurrent vte on ac in patient with established jak2-positive mpn — escalate to indefinite ac + cytoreduction optimization.
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| apixaban | 10 mg BID × 7 d → 5 mg BID full-dose; 2.5 mg BID extended-phase after first 6 mo if continuing indefinite | PO | BID × ≥3 months minimum, indefinite if splanchnic / recurrent / persistent high risk | AMPLIFY (Agnelli NEJM 2013 PMID 23808982) — apixaban first-line; Barbui Blood Adv 2021 (PMID 33591542) — DOAC acceptable in MPN VTE; AMPLIFY-EXT supports 2.5 mg BID extended-phase |
| rivaroxaban | 15 mg BID × 21 d → 20 mg daily; 10 mg daily extended-phase after first 6 mo if continuing indefinite | PO | BID then daily ≥3 months, indefinite per criteria | EINSTEIN-DVT (Bauersachs NEJM 2010 PMID 21128814); Barbui 2021 acceptable in MPN |
| edoxaban | 60 mg PO daily (30 mg if CrCl 15-50, weight ≤60 kg, or with strong P-gp inhibitor) after 5-10 d LMWH bridge | PO | daily × ≥3 months, indefinite per criteria | Hokusai-VTE (Büller NEJM 2013 PMID 23991958) |
| warfarin | 5 mg daily; INR target 2-3 (target 3 if triple-positive APS per ISTH) | PO | daily ≥3 months, indefinite per criteria | TRAPS (Pengo Blood 2018 PMID 30002145) — warfarin > rivaroxaban in triple-positive APS; preferred in unstable hepatic dysfunction where DOAC clearance unpredictable |
| enoxaparin | 1 mg/kg SC BID; reduce to 1 mg/kg daily if CrCl <30 | SC | BID | ASH 2020 (PMID 33007077); ASH 2018 pregnancy (PMID 30482767) — LMWH first-line in pregnancy and as bridge during workup |
| hydroxyurea | 500 mg PO BID titrate to platelet target < 400 × 10⁹/L and Hct target | PO | BID | ELN 2024 first-line cytoreduction in PV and high-risk ET; Carobbio 2007 (PMID 17566071) — leukocytosis-driven thrombosis suppressed by HU; long-track-record safety |
| peginterferon-alfa-2a | 45–90 µg SC weekly titrate | SC | weekly | PROUD-PV / CONTINUATION-PV — interferon non-inferior to HU with potential disease-modifying allele-burden reduction; preferred in pregnancy and young patients |
| ruxolitinib | 10 mg PO BID titrate per platelets (RESPONSE protocol) | PO | BID | RESPONSE (Vannucchi NEJM 2015 PMID 25629741) — ruxolitinib superior to best available therapy in HU-resistant/intolerant PV; ELN 2024 endorsed |
| aspirin | 81 mg daily | PO | daily indefinite | ECLAP (Landolfi NEJM 2004 PMID 14702426) — low-dose ASA reduces thrombosis in PV; standard in PV and ET; HOLD if extreme thrombocytosis (> 1500) until cytoreduction lowers platelets and acquired vWS resolves |
Plan: JAK2-V617F-positive MPN VTE — acute AC + low-dose ASA + cytoreduction + phlebotomy (PV) — multi-axis regimen (ELN 2024; CYTO-PV; ECLAP; ASH 2020; Barbui 2021)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
Long-term hematology + thrombosis clinic co-management; annual bone marrow if disease progression suspected (PV / ET → PMF / leukemia transformation); pregnancy planning (interferon + LMWH preferred over hydroxyurea + DOAC / warfarin); cardiovascular risk factor optimisation; education on splenic infarct + erythromelalgia + pruritus + transformation symptoms
Guideline: ELN 2024 MPN management + ASH 2018 thrombophilia testing + ASH 2020 VTE Treatment + ACCP/CHEST 2021