Transjugular intrahepatic porto-systemic stent-shunt for therapy of bleeding esophageal varices due to extramedullary hematopoiesis in primary myelofibrosis: a case report.
BACKGROUND
Primary myelofibrosis belongs to the group of myeloproliferative syndromes. Extramedullary hematopoiesis in the liver can lead to portal hypertension.


METHODS
We report a case of a patient with life-threatening, endoscopically not treatable bleeding from esophageal varices due to extramedullary hematopoiesis of the liver that was successfully treated with placement of a transjugular intrahepatic porto-systemic stent-shunt (TIPS).


RESULTS
Therapy of variceal bleeding by TIPS insertion was successful. During a 29-month follow-up, no hepatic failure, hepatic encephalopathy, or further variceal bleeding episode occurred.


CONCLUSIONS
TIPS placement is a well-established procedure for the treatment of complications due to portal hypertension mainly due to liver cirrhosis. This report illustrates that TIPS placement can also be a promising treatment option in patients with primary myelofibrosis and portal hypertension due to extramedullary hematopoiesis.
II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar der Technischen Universität München, Germany. phillip@lrz.tum.de
Veit
V
Phillip
Hermann
H
Berger
Melanie
M
Straub
Bernd
B
Saugel
Matthias
M
Treiber
Henrik
H
Einwächter
Roland M
RM
Schmid
Wolfgang
W
Huber
D000368:Aged; D004932:Esophageal and Gastric Varices / Q000150:complications* / Q000601:surgery*; D006471:Gastrointestinal Hemorrhage / Q000150:complications* / Q000601:surgery*; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D008297:Male; D019168:Portasystemic Shunt, Transjugular Intrahepatic / Q000295:instrumentation* / Q000379:methods; D055728:Primary Myelofibrosis / Q000150:complications* / Q000601:surgery; D016896:Treatment Outcome