Esophagogastric Bleedings in Patients with Ph-Negative Myeloproliferative Neoplasms and Extrahepatic Portal Hypertension

AIM. To assess the treatment outcomes in esophagogastric bleeding patients with Ph-negative myeloproliferative neoplasms (MPN) and extrahepatic portal hypertension caused by thrombotic complications. MATERIALS & METHODS. The present prospective clinical trial performed in the surgical department of the National Research Center for Hematology from 2013 to 2024 enrolled 52 patients with Ph-negative MPNs and extrahepatic portal hypertension caused by total portal vein thrombosis. The median age of patients was 45 years (range 25–68 years). Young (≤ 40 years) and middle-aged (≤ 60 years) patients predominated: 16 (30.8 %) and 31 (59.6 %), respectively. There were 5 (9.6 %) elderly (> 60 years) patients. The V617F mutation in JAK2 was detected in 41 (78.8 %) out of 52 patients. RESULTS. Splenectomy was performed in 49 patients, 41 (78.8 %) out of them also underwent gastrotomy as well as esophageal/gastric variceal ligation. Gastrotomy was not carried out in 10 (19.2 %) out of 52 patients. Laparotomy with gastrotomy along with esophageal and gastric variceal ligation (without splenectomy) was performed in 3 (5.8 %) patients. Repeated bleedings were registered in 2 of them, which required further splenectomy. Overall incidence of postoperative complications was 21 %. Long-term rebleedings reported in 7 (13.5 %) patients were treated non-surgically. The 5-year overall survival in the total group (n = 52) was 90.1 %. At later stages of Ph-negative MPNs, 5 (9.6 %) deaths were registered as a result of the blast transformation of the disease (n = 3) and thrombohemorrhagic complications (n = 2). CONCLUSION. Patients with Ph-negative MPNs and extrahepatic portal hypertension caused by portal vein thrombosis represent most complicated cases. However, surgical procedures, such as splenectomy combined with gastrotomy and esophageal/gastric variceal ligation, considerably improve long-term treatment outcomes despite a relatively high incidence of postoperative complications (thrombotic, hemorrhagic, purulent, and inflammatory ones).