title: Clinical features of patients with Philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension.
abstract: BACKGROUND
  Portal hypertension has been reported to afflict 7% to 18% of patients with Philadelphia-negative MPNs, with complications of variceal bleeding and ascites. The clinical features and outcomes of these patients are unclear.
  
  
  METHODS
  In this multicenter retrospective study, we evaluated the clinical features of 51 patients with MPNs complicated by PHTN.
  
  
  RESULTS
  The diagnosis of underlying MPN was most frequently PV (39%) and primary MF (35%), followed by post-PV MF (18%), ET (4%), and post-ET MF (4%). Frequency of Janus Kinase 2 V617F mutation appears as expected in the underlying MPN. Thrombosis within the splanchnic circulation was prevalent in patients with polycythemia compared with other MPNs (76% vs. 26%; P = .0007).
  
  
  CONCLUSIONS
  PV and MF patients have a greater incidence of PHTN in our population, with thrombosis contributing to PHTN development in PV patients. Patients with splanchnic circulation thrombosis are potential candidates for screening for PHTN. These data might be useful for developing screening strategies for early detection of PHTN in patients with MPN.
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Matthew
authors.initials: M
authors.lastname: Yan
authors.affiliation: Division of Hematology and Medical Oncology, Mayo Clinic, Scottsdale, AZ.
authors.forename: Holly
authors.initials: H
authors.lastname: Geyer
authors.affiliation: Division of Hematology and Medical Oncology, Mayo Clinic, Scottsdale, AZ.
authors.forename: Ruben
authors.initials: R
authors.lastname: Mesa
authors.affiliation: Department of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, WI.
authors.forename: Ehab
authors.initials: E
authors.lastname: Atallah
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Jeannie
authors.initials: J
authors.lastname: Callum
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Justyna
authors.initials: J
authors.lastname: Bartoszko
authors.affiliation: Medical Oncology and Hematology, Princess Margaret Cancer Centre, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Karen
authors.initials: K
authors.lastname: Yee
authors.affiliation: Department of Biostatistics, Princess Margaret Cancer Center, Toronto, Ontario, Canada.
authors.forename: Manjula
authors.initials: M
authors.lastname: Maganti
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Florence
authors.initials: F
authors.lastname: Wong
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada; Medical Oncology and Hematology, Princess Margaret Cancer Centre, University of Toronto, Toronto, Ontario, Canada. Electronic address: vikas.gupta@uhn.ca.
authors.forename: Vikas
authors.initials: V
authors.lastname: Gupta
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D015994:Incidence; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000175:diagnosis* / Q000235:genetics; D010677:Philadelphia Chromosome; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D012189:Retrospective Studies; D013152:Splanchnic Circulation; D013927:Thrombosis / Q000453:epidemiology / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D047428:Protein Kinase Inhibitors; D053614:Janus Kinase 2
keywords: Essential thromobocythemia; JAK inhibitor; Myelofibrosis; Polycythemia vera; Thrombosis