TY - JOUR
T1 - Outcome of portal‐systemic shunt surgery for portal hypertension associated with intrahepatic obstruction in patients with agnogenic myeloid metaplasia
AU - Tefferi, Ayalew
AU - Barrett, Scott M.
AU - Silverstein, Murray N.
AU - Nagorney, David M.
PY - 1994/8
Y1 - 1994/8
N2 - 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 alone, 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 lifethreatening PHN from intrahepatic obstruction. © 1994 Wiley‐Liss, Inc.
AB - 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 alone, 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 lifethreatening PHN from intrahepatic obstruction. © 1994 Wiley‐Liss, Inc.
KW - myelofibrosis
KW - portal hypertension
KW - portosystemic shunting
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U2 - 10.1002/ajh.2830460412
DO - 10.1002/ajh.2830460412
M3 - Article
C2 - 8037184
AN - SCOPUS:0028027315
SN - 0361-8609
VL - 46
SP - 325
EP - 328
JO - American journal of hematology
JF - American journal of hematology
IS - 4
ER -