Outcome of portal-systemic shunt surgery for portal hypertension associated with intrahepatic obstruction in patients with agnogenic myeloid metaplasia

Ayalew Tefferi, S. M. Barrett, M. N. Silverstein, D. M. Nagorney

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Life-threatening portal hypertension (PHN) in patients with chronic myeloproliferative disorders may result from increased portal flow caused by marked splenomegaly or an increased resistance to portal flow from either a large vein thrombosis or an intrahepatic obstruction usually associated with agnogenic myeloid metaplasia (AMM). The former cause is correctable by splenectomy atone, whereas the latter requires portal-systemic shunt surgery. Few data exist regarding the outcome of portal-systemic shunt surgery in patients with AMM and intrahepatic obstruction. During the past 25 years, 13 patients with chronic myeloproliferative disorders underwent portal-systemic shunt surgery at our institution. The cause of PHN was intrahepatic obstruction in ten patients and hepatic vein thrombosis in three. Ten of the thirteen patients had AMM as initial diagnosis. Only one patient had intraoperative complications, and four patients had either sepsis or thrombosis during the postoperative period. Twelve patients survived the postoperative period and had a median postsurgical survival of 3 years (range, 0.25 to 19 years). The long-term complications of the operation were very few and included hepatic encephalopathy (one patient), portal vein thrombosis (one patient), and shunt occlusion (one patient). The procedure was successful in alleviating complications of PHN in all but one patient. Deterioration of hepatic function and subsequent hepatomegaly were unusual. Portal-systemic shunt surgery seems to be a useful option in patients with AMM and life-threatening PHN from intrahepatic obstruction.

Original languageEnglish (US)
Pages (from-to)325-328
Number of pages4
JournalAmerican Journal of Hematology
Volume46
Issue number4
DOIs
StatePublished - 1994

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Primary Myelofibrosis
Portal Hypertension
Myeloproliferative Disorders
Thrombosis
Postoperative Period
Budd-Chiari Syndrome
Hepatomegaly
Hepatic Encephalopathy
Splenomegaly
Intraoperative Complications
Splenectomy
Portal Vein
Veins
Sepsis

Keywords

  • Myelofibrosis
  • Portal hypertension
  • Portosystemic shunting

ASJC Scopus subject areas

  • Hematology

Cite this

Outcome of portal-systemic shunt surgery for portal hypertension associated with intrahepatic obstruction in patients with agnogenic myeloid metaplasia. / Tefferi, Ayalew; Barrett, S. M.; Silverstein, M. N.; Nagorney, D. M.

In: American Journal of Hematology, Vol. 46, No. 4, 1994, p. 325-328.

Research output: Contribution to journalArticle

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