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Severe Upper Gastrointestinal Hemorrhage in Primar ...
Severe Upper Gastrointestinal Hemorrhage in Primary Jak2+ Myelofibrosis: An Uncommon Presentation
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This case describes a 64-year-old woman with long-standing JAK2-positive myelofibrosis who presented with severe upper gastrointestinal bleeding, including hematemesis and melena during an international flight. At the outside hospital and then at Thomas Jefferson University Hospital, she was found to have marked splenomegaly, gastric varices, acute blood loss anemia, and mild lactate elevation. Her hemoglobin fell to 6.4 g/dL, requiring packed red blood cell transfusion, octreotide, and proton pump inhibitor therapy.<br /><br />CTA demonstrated massive splenomegaly and extensive gastric varices, and EGD confirmed recently bleeding gastric varices. Management was complicated by her anatomy and comorbid findings: she was not a candidate for BRTO, endoscopic cyanoacrylate injection was considered too risky because transthoracic echo showed a small patent foramen ovale, and interventional radiology advised against arterial embolization without splenectomy. Surgery recommended splenectomy, but the patient declined definitive treatment in Pennsylvania and chose patient-directed discharge to return to California.<br /><br />To reduce rebleeding risk during travel, she was discharged on carvedilol and self-administered subcutaneous octreotide, with close coordination between the inpatient team and her home hematologist-oncologist. After arrival in California, she underwent uncomplicated splenectomy and was discharged within two weeks.<br /><br />The report emphasizes that gastric varices in myelofibrosis may result from portal hypertension due to splenomegaly and/or splenic vein thrombosis. Initial treatment mirrors that for esophageal variceal bleeding, but definitive therapy depends on the underlying cause. In myelofibrosis-related cases, splenectomy, splenic artery embolization, splenic vein stenting, and JAK inhibitor therapy may be considered. The case highlights the need for multidisciplinary management involving gastroenterology, interventional radiology, surgery, and hematology/oncology.
Asset Subtitle
Nadia H. Khusro
Meta Tag
Author List
Alexis M. Metoyer, Alyssa Yeager, Nadia H. Khusro
Category
Clinical Vignettes
Concept
Primary Myelofibrosis
Concept
Portal Hypertension
Concept
Gastric Varices
Concept
Gastric Variceal Bleeding
Concept
Splenomegaly
Distinguished
Non-Finalist
Presenter Organization
Thomas Jefferson University Hospital
Presenting Author
Nadia H. Khusro
Track
Adult
Keywords
myelofibrosis
gastric varices
upper gastrointestinal bleeding
splenomegaly
hematemesis
melena
splenectomy
portal hypertension
octreotide
JAK2-positive
Primary Myelofibrosis
Portal Hypertension
Gastric Varices
Gastric Variceal Bleeding
Splenomegaly
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