A 46 years old male patient, who is a known T2 diabetes mellitus (DM) (poorly controlled) patient over the last 5 years on glimepiride and metformin, was referred to our OPD after he presented with abdominal pain of one-week duration. Physical examination was unremarkable. Laboratory studies revealed ALP was four times raised (1143 IU), hypoalbuminemia (3.1 gm), and thrombocytosis (548, 000/l-684,000/l). His fasting blood level was in the range of 199-234 gm/dl. Otherwise, laboratory studies were in the normal range. On abdominal ultrasound, the liver has a homogenous echo pattern with smooth contours, the portal vein is obliterated with periportal collaterals, and the spleen is enlarged (15.8 cm). The endoscopic evaluation revealed multiple tense tortuous lower third varices with diffuse gastric mucosal congestion, for which EVL was done. The patient was admitted and glycemic control was optimized. Warfarin, propranolol, and diuretics were commenced upon discharge. Seven months after initiation of anticoagulation the patient presented with hematemesis and melena (last INR- not known then). At presentation, his vitals were stable. Physical examination was unremarkable. CBC was normal (WBC -9200/l (74.4%) Hgb -15.7gm/dl, PLT - 578,000/l). ALP was two times raised (84 IU). Warfarin was discontinued. Therapeutic EVL was done and the patient was discharged. Upon further evaluation abroad, JAK 2 mutation was identified. However, anticoagulation was not resumed.
Updated abdominal ultrasound revealed worsening of splenomegaly (16.7 cm). Abdominal CT identified chronic portal and spleno-mesenteric vein thrombosis (Figure 5) with cavernous transformation and splenomegaly with Portal hypertensive biliopathy (Figure 6). TB was raised (1.5 mg/dl -2.2 mg/dl). PLT was persistently elevated. Peripheral morphology revealed thrombocytosis. He has taken several sessions of prophylactic EVL. With the diagnosis of non-cirrhotic portal hypertension secondary to portal vein thrombosis secondary to high-risk PV, he was put on propranolol, hydroxyurea, and aspirin, with intermittent prophylactic sessions of EVL.  
During the course of his treatment, he had several admissions for prophylactic and therapeutic EVL. Upon follow up his hematocrit level was significantly raised (52.9%) which necessitated two sessions of 1-unit phlebotomy, one month apart. His post-phlebotomy hematocrit level dropped to 48.1%. Currently, his symptoms are well controlled.