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

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.