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Clin Lymphoma Myeloma Leuk. 2015 Jan;15(1):e1-5. doi: 10.1016/j.clml.2014.04.004. Epub 2014 Jun 11.

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

Author information

1
Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
2
Division of Hematology and Medical Oncology, Mayo Clinic, Scottsdale, AZ.
3
Department of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, WI.
4
Medical Oncology and Hematology, Princess Margaret Cancer Centre, University of Toronto, Toronto, Ontario, Canada.
5
Department of Biostatistics, Princess Margaret Cancer Center, Toronto, Ontario, Canada.
6
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.

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.

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

PMID:
25027569
PMCID:
PMC4361182
DOI:
10.1016/j.clml.2014.04.004
[Indexed for MEDLINE]
Free PMC Article

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