Contributions
Abstract: PB2314
Type: Publication Only
Background
PMFoften causes SVT.Data regarding outcome of this subset of patients, mainly if treated with VKAplus ruxolitinib,are very few.
Aims
Aim of this case series is to analyze if use of ruxolitinib+VKA is safe and effective in treatment of patients withPMF with IPSS INT-2.
Methods
This study is a retrospective study.4female patients, median age47(R35-55), withPMF INT-2 and with SVT(2portal,2mesenteric+splenic+portal),median Hb11.5g/dl(R11-12.5), PLT90000/mcl(R70000-100000), WBC10000/mcl(R4000-11000), peripheral blood blasts 1%(R1-2), 1 patient heterozygous for factor V Leiden received ruxolitinib20mg/day+warfarin. All patients were Jak-2 mutated(GROUP1).In an historical cohort 6patients 2male,4female median age60(R45-65), 3PV,1ET,1PMF, 1 paroxysmal nocturnal hemoglobinuria and with SVT(4portal,2splenic+portal),median Hb12.5g/dl(R10-13.5), PLT150000/mcl(R110000-200000), WBC8000/mcl(R6000-10000), peripheral blood blasts 0%(R0-1), 1 patient heterozygous for factor V Leiden, 1 for prothrombin G20210, received Hydroxyurea 1000 mg(R500-1500)/day+warfarin. 3 patients were Jak-2 mutated(GROUP2).In an other historical cohort 6patients 4male,2female median age60(R55-70), 3liver cirrhosis,3solid cancer and with SVT(5portal,1splenic+portal),median Hb11.5g/dl(R9-12.5), PLT100000/mcl(R90000-130000), WBC4000/mcl(R2000-9000), peripheral blood blasts 0%,received only warfarin. 1 patient was Jak-2 mutated(GROUP3).All patients received an abdominal vascular doppler echography, C reactive protein(CRP) and D-Dimers(DD) mesuration at0, 3and6 months of follow-up. In patients receiving ruxolitinib circulating endothelial cells(CEC) were measured at same time (in flow cytometry with BDantibody CD146).
Results
Patients of GROUP1 showed a complete resolution of SVT in 2 cases and a partial portal recanalization in 2 cases after 3 months, without any thrombosis relapse or progression after 6 months; patients of GROUP2 showed a complete resolution of SVT in 2 cases, a partial portal recanalization in 2 cases and no resolution in 2 cases only after 6 months; patients of GROUP3 showed a partial portal recanalization in 3 cases, no resolution in 2 cases and 1 progression only after 6 months.MedianCRP after0, 3and6 months(mg/l) was respectively inGROUP1 27(R18-33),8(R3-10),6(R3-7),inGROUP2 30(R20-35),18(R12-22),10(R7-15),in GROUP3 38(R28-40),35(R27-42),33(R22-35).
MedianDD after0, 3and6 months(ng/ml) were respectively inGROUP1 6000(R4500-7000),500(R300-1000),130(R200-300),inGROUP2 4500(R3200-5500),900(R850-2100),400(R350-800),in GROUP3 2800(R1300-3200),4700(R3700-4900),3500(R3000-4800).
MedianCEC/ml inGROUP1 after0, 3and6 months(mg/l) were respectively 1500(R800-5500),800(R600-1800),500(R400-1200).
InGROUP1 all patients showed reduction of spleen dimension and improuvement of systemic symptoms, InGROUP2 3 patients showed reduction of spleen dimension and0 improuvement of systemic symptoms, InGROUP3 no patients showed reduction of spleen dimension and improuvement of systemic symptoms.
No patient showed side effects treatment related.
Conclusion
Ruxolitinib and VKA is safe and effective and shows a faster recanalization and an antinflammatory effect in patients with PMF with SVT. These results needs confirmation on a larger cohort of patients.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Endothelial cell, Myelofibrosis, Ruxolitinib, Thrombosis
Abstract: PB2314
Type: Publication Only
Background
PMFoften causes SVT.Data regarding outcome of this subset of patients, mainly if treated with VKAplus ruxolitinib,are very few.
Aims
Aim of this case series is to analyze if use of ruxolitinib+VKA is safe and effective in treatment of patients withPMF with IPSS INT-2.
Methods
This study is a retrospective study.4female patients, median age47(R35-55), withPMF INT-2 and with SVT(2portal,2mesenteric+splenic+portal),median Hb11.5g/dl(R11-12.5), PLT90000/mcl(R70000-100000), WBC10000/mcl(R4000-11000), peripheral blood blasts 1%(R1-2), 1 patient heterozygous for factor V Leiden received ruxolitinib20mg/day+warfarin. All patients were Jak-2 mutated(GROUP1).In an historical cohort 6patients 2male,4female median age60(R45-65), 3PV,1ET,1PMF, 1 paroxysmal nocturnal hemoglobinuria and with SVT(4portal,2splenic+portal),median Hb12.5g/dl(R10-13.5), PLT150000/mcl(R110000-200000), WBC8000/mcl(R6000-10000), peripheral blood blasts 0%(R0-1), 1 patient heterozygous for factor V Leiden, 1 for prothrombin G20210, received Hydroxyurea 1000 mg(R500-1500)/day+warfarin. 3 patients were Jak-2 mutated(GROUP2).In an other historical cohort 6patients 4male,2female median age60(R55-70), 3liver cirrhosis,3solid cancer and with SVT(5portal,1splenic+portal),median Hb11.5g/dl(R9-12.5), PLT100000/mcl(R90000-130000), WBC4000/mcl(R2000-9000), peripheral blood blasts 0%,received only warfarin. 1 patient was Jak-2 mutated(GROUP3).All patients received an abdominal vascular doppler echography, C reactive protein(CRP) and D-Dimers(DD) mesuration at0, 3and6 months of follow-up. In patients receiving ruxolitinib circulating endothelial cells(CEC) were measured at same time (in flow cytometry with BDantibody CD146).
Results
Patients of GROUP1 showed a complete resolution of SVT in 2 cases and a partial portal recanalization in 2 cases after 3 months, without any thrombosis relapse or progression after 6 months; patients of GROUP2 showed a complete resolution of SVT in 2 cases, a partial portal recanalization in 2 cases and no resolution in 2 cases only after 6 months; patients of GROUP3 showed a partial portal recanalization in 3 cases, no resolution in 2 cases and 1 progression only after 6 months.MedianCRP after0, 3and6 months(mg/l) was respectively inGROUP1 27(R18-33),8(R3-10),6(R3-7),inGROUP2 30(R20-35),18(R12-22),10(R7-15),in GROUP3 38(R28-40),35(R27-42),33(R22-35).
MedianDD after0, 3and6 months(ng/ml) were respectively inGROUP1 6000(R4500-7000),500(R300-1000),130(R200-300),inGROUP2 4500(R3200-5500),900(R850-2100),400(R350-800),in GROUP3 2800(R1300-3200),4700(R3700-4900),3500(R3000-4800).
MedianCEC/ml inGROUP1 after0, 3and6 months(mg/l) were respectively 1500(R800-5500),800(R600-1800),500(R400-1200).
InGROUP1 all patients showed reduction of spleen dimension and improuvement of systemic symptoms, InGROUP2 3 patients showed reduction of spleen dimension and0 improuvement of systemic symptoms, InGROUP3 no patients showed reduction of spleen dimension and improuvement of systemic symptoms.
No patient showed side effects treatment related.
Conclusion
Ruxolitinib and VKA is safe and effective and shows a faster recanalization and an antinflammatory effect in patients with PMF with SVT. These results needs confirmation on a larger cohort of patients.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Endothelial cell, Myelofibrosis, Ruxolitinib, Thrombosis