Clinical features of patients with Philadelphia-negative myeloproliferative neoplasms complicated by portal hypertension. Clin Lymphoma Myeloma Leuk 2015 Jan;15(1):e1-5
Date
07/17/2014Pubmed ID
25027569Pubmed Central ID
PMC4361182DOI
10.1016/j.clml.2014.04.004Scopus ID
2-s2.0-84934440451 (requires institutional sign-in at Scopus site) 22 CitationsAbstract
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.
Author List
Yan M, Geyer H, Mesa R, Atallah E, Callum J, Bartoszko J, Yee K, Maganti M, Wong F, Gupta VAuthor
Ehab L. Atallah MD Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultAged
Aged, 80 and over
Female
Follow-Up Studies
Humans
Hypertension, Portal
Incidence
Janus Kinase 2
Male
Middle Aged
Mutation
Myeloproliferative Disorders
Philadelphia Chromosome
Portasystemic Shunt, Transjugular Intrahepatic
Prognosis
Protein Kinase Inhibitors
Retrospective Studies
Splanchnic Circulation
Thrombosis
Treatment Outcome