Abstract Number: PB2250
Meeting: ISTH 2020 Congress
Background: Splanchnic vein thromboses (SVT) often cause Budd-Chiari syndrome (BCS). JAK2-MPN is the most common cause of SVT/BCS. Endothelial cells harboring JAK2 mutations have been demonstrated in patients with BCS which may contribute to the overall prothrombotic propensity.
Aims: This study attempts to describe the epidemiology and clinicopathology of latent JAK2-MPN (LMPN), where JAK2 mutation is detected without clinical evidence of MPN, versus active JAK2-MPN (AMPN) where JAK2+ MPN is present, in patients with SVT/ BCS.
Methods: We compiled a pooled database of 90 cases with SVT/BCS. Kaplan-Meier survival curves, Cox proportional-hazards model, and Log-rank tests were used to assess the influence of demographic and clinical factors on survival.
Results: The SVT/BCS cohort consisted of 65% LMPN and 35% AMPN with median ages of 39 and 42 years respectively. The peak incidence of SVT/BCS seems to occur between ages 36-48 for LMPN and 24-36 for AMPN. Females were twice more predominant than males. The AMPN group consisted of 41% Polycythemia Vera (PV), 22% Essential Thrombocythemia, 28% Myelofibrosis (MF) and 3% others. There was no difference in the type of the splanchnic vein involvement or the number of coexisting acquired or inherited conditions between the two groups. Survival of patients with BCS did not differ by their MPN status or sex. 21% of LMPN progressed to AMPN (57% ET, 21.5% PV, and 21.5% MF). The median time to progression was 55 months among those that progressed. While the median time to progression was longer for males (63 vs 39 months) this difference did not reach statistical significance.
Conclusions: This study describes for the first time the epidemiologic and clinical characteristics of patients with SVT/BSC by their MPN status. It reveals that the majority of LMPN do not progress to AMPN, indicating that LMPN prothrombotic propensity is independent of the hematologic AMPN clinical expression.
To cite this abstract in AMA style:Haddad PA, Hammoud D, Gallagher K. Epidemiology and Clinicopathology of Latent versus Active JAK2-Myeloproliferative Neoplasms (MPN) in Splanchnic Venous Thrombosis [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/epidemiology-and-clinicopathology-of-latent-versus-active-jak2-myeloproliferative-neoplasms-mpn-in-splanchnic-venous-thrombosis/. Accessed November 26, 2020.
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