Myeloproliferative neoplasms and splanchnic vein thrombosis: Contemporary diagnostic and therapeutic strategies

Abstract

Myeloproliferative neoplasms (MPNs) are the most common etiologies of primary splanchnic vein thrombosis, present in almost forty percent of patients with Budd‐Chiari syndrome or portal vein thrombosis. Diagnosis of MPNs can be difficult in these patients because key characteristics, such as elevated blood cell counts and splenomegaly, are confounded by portal hypertension or bleeding complications. In recent years, diagnostic tools have improved to provide more accurate diagnosis and classification of MPNs. Although bone marrow biopsy findings remain a major diagnostic criterion, molecular markers are playing an increasing role not only in diagnosis but also in better estimating prognosis. Therefore, though screening for JAK2V617F mutation should be the starting point of the diagnostic workup performed in all patients with splanchnic vein thrombosis, a multidisciplinary approach is needed to accurately diagnose the subtype of myeloproliferative neoplasm, recommend the useful additional tests (bone marrow biopsy, search for an additional mutation using targeted next‐generation sequencing), and suggest the best treatment strategy. Indeed, providing a specific expert care pathway for patients with splanchnic vein thrombosis and underlying myeloproliferative neoplasm is crucial to determine the optimal management to reduce the risk of both hematological and hepatic complications.

Intestinal Obstruction as Initial Presentation of Idiopathic Portal and Mesenteric Venous Thrombosis: Diagnosis, Management, and Literature Review

“…Sometimes, there are no obvious factors that can be identified as the cause of the thrombotic event. Since idiopathic PVTs are uncommon, more research is necessary to determine the best course of treatment, including anticoagulation and follow-up [55].…”

Splanchnic Vein Thrombosis: The State-of-the-Art on Anticoagulant Treatment

“…40 Other driver mutations causing MPNs, such as calreticulin (CALR) and thrombopoietin receptor (MPL) mutations, are rarely found in SVT patients (1%). Next-generation sequencing has been utilized in more recent studies to detect nondriver mutations 41 ; however, the association of similar mutations with thrombotic risk is still unclear. 42,43 Another rare hematological disorder associated with SVT is paroxysmal nocturnal hemoglobinuria (PNH).…”

“…15 It is debatable whether a combination of antiplatelets and anticoagulants should be prescribed for MPN-associated SVT, because it might increase the already relevant risk of gastrointestinal bleeding. 41 In the study by Sant'Antonio et al, approximately 14% of MPN patients with SVT received antiplatelet treatment, either alone or in association with anticoagulants. However, at multivariable analysis, major bleeding was mainly related to the presence of gastroesophageal varices and not to the type of treatment.…”

The clinical significance of TAT, PIC, TM, and t-PAIC in vascular events of BCR/ABL-negative myeloproliferative neoplasms

“…MPN are the most common etiologies of primary splanchnic vein thrombosis (SVT), including mesenteric, splenic, or portal vein thrombosis (PVT), with about 30–40% of SVT being caused by MPN [ 5 ]. All types of MPN have been reported to cause vein thrombosis, though the most frequent appears to be PV, followed by ET and PMF [ 6 ]. The primary goal of treatment, especially those with PV and ET, is to prevent thrombotic complications typically through antiplatelet therapy and/or cytoreduction [ 6 ].…”

“…All types of MPN have been reported to cause vein thrombosis, though the most frequent appears to be PV, followed by ET and PMF [ 6 ]. The primary goal of treatment, especially those with PV and ET, is to prevent thrombotic complications typically through antiplatelet therapy and/or cytoreduction [ 6 ].…”