Wiest, Reiner and Strauch, U. and Wagner, H. and Strotzer, M. and Woenckhaus, M. and Schroeder, G. and Schoelmerich, Juergen and Lock, G. (2004) A patient with myelofibrosis complicated by refractory ascites and portal hypertension: to tips or not to tips? A case report with discussion of the mechanism of ascites formation. SCANDINAVIAN JOURNAL OF GASTROENTEROLOGY, 39 (4). pp. 389-394. ISSN 0036-5521
Full text not available from this repository. (Request a copy)Abstract
In patients with myelofibrosis, clinically significant portal hypertension is known to be predominantly presinusoidal; however, the exact mechanisms are still controversial. The pathophysiology is particularly enigmatic in those patients without histological and angiographic evidence of significant intra- or extrahepatic obstruction to portal blood flow, respectively. Moreover, ascites formation has been reported in such cases, but in general is rare in presinusoidal portal hypertension. Here we present such a patient in which ascites developed even in the presence of unchanged serum protein levels (oncotic pressure) and was refractory to sodium restricted diet and high-dose diuretic treatment. A discussion on the parameters influencing fluid exchange and ascites formation particularly emphasizing the potential importance of the hyperdynamic circulation in this case is given. Finally, the patient was treated by implanting a trans-jugular intrahepatic shunt (TIPS), exerting a diuretic effect sufficient enough to avoid re-formation of ascites for several months. However, ascites re-accumulated potentially due to the appearance of ectopic peritoneal myeloid metaplasia and the patient died soon afterwards. In conclusion, TIPS may be considered as rescue management for refractory ascites secondary to portal hypertension, but caution in respect to the presence and/or development of peritoneal or other ectopic haematopoesis has to be taken.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | AGNOGENIC MYELOID METAPLASIA; INTRAHEPATIC PORTOSYSTEMIC SHUNT; EXTRAMEDULLARY HEMATOPOIESIS; MYELOPROLIFERATIVE-SYNDROME; IDIOPATHIC MYELOFIBROSIS; NATURAL HISTORY; BLOOD FLOW; PERITONEAL; MEGAKARYOCYTES; CIRCULATION; ascites; myelofibrosis; portal hypertension; portosystemic shunt |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Innere Medizin I Medicine > Lehrstuhl für Pathologie Medicine > Lehrstuhl für Röntgendiagnostik |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 26 Jul 2021 07:00 |
| Last Modified: | 26 Jul 2021 07:00 |
| URI: | https://pred.uni-regensburg.de/id/eprint/37848 |
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