[A rare cause of variceal bleeding]

Area of Science:

• Hematology
• Gastroenterology

Background:

• Agnogenic myeloid metaplasia (AMM) is a myeloproliferative neoplasm characterized by myelofibrosis, osteosclerosis, and pancytopenia.
• Portal hypertension secondary to extramedullary hematopoiesis is a known complication of AMM, though rarely clinically significant.

Observation:

• A 63-year-old patient presented with variceal bleeding, initially suspected as liver cirrhosis due to splenomegaly.
• Further investigation revealed extramedullary hematopoiesis and diagnosed Agnogenic myeloid metaplasia.

Findings:

• The patient's variceal bleeding was managed with endoscopic therapy.
• Cytoreductive therapy and a splenorenal shunt were administered, leading to remission.

Implications:

• Agnogenic myeloid metaplasia is an uncommon etiology of portal hypertension and variceal bleeding.
• Awareness of this association is crucial for clinicians to ensure timely diagnosis and management.
• Effective treatment of AMM and portal hypertension can result in favorable long-term outcomes.