Budd-Chiari syndrome caused by hepatic vein thrombosis in a patient with myeloproliferative disorder

被引:5
作者
Usui, T [1 ]
Kitano, K [1 ]
Midorikawa, T [1 ]
Yoshizawa, K [1 ]
Kobayashi, H [1 ]
Tanaka, E [1 ]
Matsunami, H [1 ]
Kawasaki, S [1 ]
Kiyosawa, K [1 ]
机构
[1] SHINSHU UNIV,SCH MED,DEPT INTERNAL MED 2,MATSUMOTO,NAGANO 390,JAPAN
关键词
latent myeloproliferative disorder; liver transplantation;
D O I
10.2169/internalmedicine.35.871
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
We report a 24-year-old Japanese female hospitalized with jaundice and ascites. She exhibited hepatosplenomegaly, severe liver dysfunction, and slight polycythemia with an increase in serum levels of beta-thromboglobin and platelet factor 4. Bone marrow was hypercellular with an increase in progenitor cells. The aggregation response of platelets to ADP and to collagen was markedly increased. Venography revealed narrowed hepatic veins with 'spider web' sign. Liver biopsy revealed hepatic congestion. Budd-Chiari syndrome was diagnosed, and was thought to be due to thrombosis related to myeloproliferative disorder. Liver transplant was successful in relieving symptoms.
引用
收藏
页码:871 / 875
页数:5
相关论文
共 18 条
[11]  
Kretz R, 1902, ERGEBALLG PATHOL ANT, V82, P473
[12]   BUDD-CHIARI SYNDROME - ETIOLOGY, DIAGNOSIS AND MANAGEMENT [J].
MITCHELL, MC ;
BOITNOTT, JK ;
KAUFMAN, S ;
CAMERON, JL ;
MADDREY, WC .
MEDICINE, 1982, 61 (04) :199-218
[13]  
PAGLIUCA A, 1990, Q J MED, V76, P981
[14]  
Pleasants JH, 1911, JOHNS HOPKINS HOSP R, V16, P363
[15]  
SHERLOCK S, 1981, DIS LIVER BILIARY, P213
[16]   LIFE-THREATENING THROMBOTIC AND HEMORRHAGIC PROBLEMS ASSOCIATED WITH SILENT MYELOPROLIFERATIVE DISORDERS [J].
VADHER, BD ;
MACHIN, SJ ;
PATTERSON, KG ;
SUKHU, C ;
WALKER, H .
BRITISH JOURNAL OF HAEMATOLOGY, 1993, 85 (01) :213-216
[17]   PRIMARY MYELOPROLIFERATIVE DISORDER AND HEPATIC VEIN-THROMBOSIS - A PROSPECTIVE-STUDY OF ERYTHROID COLONY FORMATION INVITRO IN 20 PATIENTS WITH BUDD-CHIARI SYNDROME [J].
VALLA, D ;
CASADEVALL, N ;
LACOMBE, C ;
VARET, B ;
GOLDWASSER, E ;
FRANCO, D ;
MAILLARD, JN ;
PARIENTE, EA ;
LEPORRIER, M ;
RUEFF, B ;
MULLER, O ;
BENHAMOU, JP .
ANNALS OF INTERNAL MEDICINE, 1985, 103 (03) :329-334
[18]   HEPATIC VEIN-THROMBOSIS IN PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA - A SPECTRUM FROM ASYMPTOMATIC OCCLUSION OF HEPATIC VENULES TO FATAL BUDD-CHIARI SYNDROME [J].
VALLA, D ;
DHUMEAUX, D ;
BABANY, G ;
HILLON, P ;
RUEFF, B ;
ROCHANT, H ;
BENHAMOU, JP .
GASTROENTEROLOGY, 1987, 93 (03) :569-575