Clinical features of patients with philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension

Matthew Yan, Holly Geyer, Ruben Mesa, Ehab Atallah, Jeannie Callum, Justyna Bartoszko, Karen Yee, Manjula Maganti, Florence Wong, Vikas Gupta

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Portal hypertension (PHTN) is an understudied complication of Philadelphia-negative myeloproliferative neoplasms (MPNs). We restrospectively reviewed 51 MPN patients with PHTN and found that patients with polycythemia vera (PV) and myelofibrosis (MF) are disproportionately affected by PHTN compared with essential thrombocythemia (ET). The etiology of PHTN appears to be related to splanchnic circulation thrombosis in PV and splenomegaly in MF. 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. Patients and 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). Conclusion: 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.

Original languageEnglish (US)
Pages (from-to)e1-e5
JournalClinical Lymphoma, Myeloma and Leukemia
Volume15
Issue number1
DOIs
StatePublished - 2015

Fingerprint

Portal Hypertension
Polycythemia Vera
Primary Myelofibrosis
Neoplasms
Splanchnic Circulation
Essential Thrombocythemia
Thrombosis
Janus Kinase 2
Polycythemia
Splenomegaly
Ascites
Multicenter Studies
Retrospective Studies
Hemorrhage
Mutation

Keywords

  • Essential thromobocythemia
  • JAK inhibitor
  • Myelofibrosis
  • Polycythemia vera
  • Thrombosis

ASJC Scopus subject areas

  • Cancer Research
  • Hematology
  • Oncology

Cite this

Clinical features of patients with philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension. / Yan, Matthew; Geyer, Holly; Mesa, Ruben; Atallah, Ehab; Callum, Jeannie; Bartoszko, Justyna; Yee, Karen; Maganti, Manjula; Wong, Florence; Gupta, Vikas.

In: Clinical Lymphoma, Myeloma and Leukemia, Vol. 15, No. 1, 2015, p. e1-e5.

Research output: Contribution to journalArticle

Yan, M, Geyer, H, Mesa, R, Atallah, E, Callum, J, Bartoszko, J, Yee, K, Maganti, M, Wong, F & Gupta, V 2015, 'Clinical features of patients with philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension', Clinical Lymphoma, Myeloma and Leukemia, vol. 15, no. 1, pp. e1-e5. https://doi.org/10.1016/j.clml.2014.04.004
Yan, Matthew ; Geyer, Holly ; Mesa, Ruben ; Atallah, Ehab ; Callum, Jeannie ; Bartoszko, Justyna ; Yee, Karen ; Maganti, Manjula ; Wong, Florence ; Gupta, Vikas. / Clinical features of patients with philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension. In: Clinical Lymphoma, Myeloma and Leukemia. 2015 ; Vol. 15, No. 1. pp. e1-e5.
@article{600c8fef33c944a9bf7b3ff608a0c240,
title = "Clinical features of patients with philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension",
abstract = "Portal hypertension (PHTN) is an understudied complication of Philadelphia-negative myeloproliferative neoplasms (MPNs). We restrospectively reviewed 51 MPN patients with PHTN and found that patients with polycythemia vera (PV) and myelofibrosis (MF) are disproportionately affected by PHTN compared with essential thrombocythemia (ET). The etiology of PHTN appears to be related to splanchnic circulation thrombosis in PV and splenomegaly in MF. 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. Patients and 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). Conclusion: 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.",
keywords = "Essential thromobocythemia, JAK inhibitor, Myelofibrosis, Polycythemia vera, Thrombosis",
author = "Matthew Yan and Holly Geyer and Ruben Mesa and Ehab Atallah and Jeannie Callum and Justyna Bartoszko and Karen Yee and Manjula Maganti and Florence Wong and Vikas Gupta",
year = "2015",
doi = "10.1016/j.clml.2014.04.004",
language = "English (US)",
volume = "15",
pages = "e1--e5",
journal = "Clinical Lymphoma, Myeloma and Leukemia",
issn = "2152-2669",
publisher = "Cancer Media Group",
number = "1",

}

TY - JOUR

T1 - Clinical features of patients with philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension

AU - Yan, Matthew

AU - Geyer, Holly

AU - Mesa, Ruben

AU - Atallah, Ehab

AU - Callum, Jeannie

AU - Bartoszko, Justyna

AU - Yee, Karen

AU - Maganti, Manjula

AU - Wong, Florence

AU - Gupta, Vikas

PY - 2015

Y1 - 2015

N2 - Portal hypertension (PHTN) is an understudied complication of Philadelphia-negative myeloproliferative neoplasms (MPNs). We restrospectively reviewed 51 MPN patients with PHTN and found that patients with polycythemia vera (PV) and myelofibrosis (MF) are disproportionately affected by PHTN compared with essential thrombocythemia (ET). The etiology of PHTN appears to be related to splanchnic circulation thrombosis in PV and splenomegaly in MF. 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. Patients and 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). Conclusion: 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.

AB - Portal hypertension (PHTN) is an understudied complication of Philadelphia-negative myeloproliferative neoplasms (MPNs). We restrospectively reviewed 51 MPN patients with PHTN and found that patients with polycythemia vera (PV) and myelofibrosis (MF) are disproportionately affected by PHTN compared with essential thrombocythemia (ET). The etiology of PHTN appears to be related to splanchnic circulation thrombosis in PV and splenomegaly in MF. 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. Patients and 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). Conclusion: 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.

KW - Essential thromobocythemia

KW - JAK inhibitor

KW - Myelofibrosis

KW - Polycythemia vera

KW - Thrombosis

UR - http://www.scopus.com/inward/record.url?scp=84934440451&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84934440451&partnerID=8YFLogxK

U2 - 10.1016/j.clml.2014.04.004

DO - 10.1016/j.clml.2014.04.004

M3 - Article

VL - 15

SP - e1-e5

JO - Clinical Lymphoma, Myeloma and Leukemia

JF - Clinical Lymphoma, Myeloma and Leukemia

SN - 2152-2669

IS - 1

ER -