Matthew Yan1, Holly Geyer2, Ruben Mesa2, Ehab Atallah3, Jeannie Callum1, Justyna Bartoszko1, Karen Yee4, Manjula Maganti5, Florence Wong1, Vikas Gupta6. 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.
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.
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