Prekchha Jha, MBBS, Nishant Aggarwal, MBBS, Rabin Neupane, MBBS, Mihaela Batke, MD Corewell Health, Royal Oak, MI
Introduction: Polycythemia Vera (PV) is a myeloproliferative neoplasm categorized by the presence of elevated red blood cell mass. It causes an increased risk of thrombosis but is not usually linked with gastrointestinal bleed (GIB). Portal hypertension is a common cause of esophageal variceal bleeding in patients with cirrhotic liver. However, Polycythemia Vera can lead to noncirrhotic portal hypertension (NCPH). Here we present a case of recurrent upper GIB because of NCPH due to polycythemia Vera without thrombotic complications.
Case Description/Methods: A 71-year-old male presented to the Emergency Department (ED) due to 2 weeks of melena. He had a similar episode four months prior to this presentation. He was diagnosed with PV about 20 years ago, which eventually transformed to myelofibrosis in 2022 with positive JAK2. In the ED, labs were notable for hemoglobin 6.3 g/dl(normal 13.5-17g/dl). He was initially managed with 2 units of pRBC transfusion, IV fluids, IV PPI and octreotide drip. CT scan of the abdomen was notable for splenomegaly. Doppler Ultrasound of the liver showed patent hepatic vasculature without any evidence of thrombosis. A prior MRI elastography done three months ago had shown multiple enhancing bilobar nodules suggestive of multiple focal nodular hyperplasia versus regenerative nodular hyperplasia. At the current admission, the patient underwent upper endoscopy which revealed grade II varices in the lower esophagus which were successfully banded. Following this, his hemoglobin stabilized along with the improvement of symptoms. The patient was subsequently discharged with oral PPI and repeat endoscopy was planned for six weeks for variceal screening.
Discussion: Bleeding esophageal varices is a common entity that is found in cirrhotic patients due to portal hypertension. Additionally, thrombosis of liver vasculature may lead to NCPH. Myeloproliferative disorders predispose to intrahepatic thrombotic complications. However, as evidenced by the case above, patients with PV can have NCPH in absence of thrombosis. In such patients, a nodular transformation along with increased portal flow due to splenomegaly could be significant contributory factors. Thus, in conclusion, in patients with myeloproliferative disorders who present with GIB, it is important to consider NCPH even in absence of thrombotic complications. This will help in an early diagnosis and intervention.
Prekchha Jha indicated no relevant financial relationships.
Nishant Aggarwal indicated no relevant financial relationships.
Rabin Neupane indicated no relevant financial relationships.
Mihaela Batke indicated no relevant financial relationships.
Prekchha Jha, MBBS, Nishant Aggarwal, MBBS, Rabin Neupane, MBBS, Mihaela Batke, MD. P4001 - Polycythemia Vera: An Unusual Cause of Gastrointestinal Bleed, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.