[A rare cause of variceal bleeding].

Background: Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disease, which is characterized by myelofibrosis, osteosclerosis and pancytopenia. Affected patients frequently develop portal hypertension secondary to extramedullary hematopoiesis, which rarely becomes clinically relevant.

Case Report: We here report on a 63-year-old patient with a first presentation of variceal bleeding. The patient was diagnosed with portal hypertension and due to marked splenomegaly, liver cirrhosis was suspected. Subsequently, an extramedullary hematopoiesis and AMM could be diagnosed. The variceal bleeding was initially treated endoscopically. The patient has received cytoreductive therapy and a splenorenal shunt, which has led to remission and long-term survival.

Conclusion: Agnogenic myeloid metaplasia is a rare cause of portal hypertension and may present with variceal bleeding. Clinicians should be aware of this rare cause of portal hypertension, as treatment of the underlying disease with cytoreductive therapy and appropriate management of portal hypertension may lead to long-term survival.