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Hepatic vein webs and short-length stenoses in patients with primary myeloproliferative disorders
DECREASED BILIRUBIN CONJUGATION CAPACITY IN PEDIATRIC PATIENTS WITH ~-THALASSEMIA
Ullrich,D+;Grotefendt,M;Sieq,A;Bircher,J and W.Schr6ter + Departments of Clinical Pharmacology and Pediatrics+; University of G6ttingen;FRG. In patients with homozygous ~-thalassemia (thal) hyperbilirubinemia has been observed, even when erythropoesis had been suppressed by a high-frequency transfusion regimen and biochemical evidences for hepatic diseases were absent. It appeared pertinent therefore to further investigate the origin of this hyperbilirubinemia. Methods: 15 patients (6 males, 9 females, aged 1-23 y) with homozygous 5-±hal and 1 patient (male) with sicklecell-thal were studied: hemoglobin was 9.7±2.0 g/dl (mean ± ISD); haptoglobin 0.79±0.72 ng/ml; GPT 43±34 U/I; serum ferritin 2057±1225 ng/ml and density on CT-scan of the liver was 86.6±13.5 Houndsfield U (n:<80). Patients had neither signs for hepatitis B nor evidence for cirrhosis. 14 healthy infants (5 males, 9 females; 3-18 years) and 26 adults (14 males, 12 females, 19-63 years) served as controls. Total bilirubin was measured by diazo-method, bilirubin-conjugates were determined by alkaline methanolysis followed by thin-layer-chromatography. Results: In patients with ±hal total bilirubin was 24.1±22.6 pmol/l compared to 6.9±4.2 (p
HEPATIC VEIN WEBS AND SHORT-LENGTH STENOSES IN PATIENTS WITH PRI~MRY ['~ELOPROLIFERATIVE DISORDERS D. Valla, Y. Menu, M. Morcelet, G. Molas, J. Bel~hiti, H6pital Beaujon, 92118 Clichy, France
B. Rueff, and JP. Benhamou..
In some patients with Budd-Chiari syndrome, hepatic venous outflow block is caused by fibrous, short-length stenoses or webs involving the hepatic vein ostia or their vicinity. Such lesions have been variously attributed to a congenital, inflammatory, or thrombotic origin. We report the cases of 8 patients with such lesions among 56 consecutive patients with Budd-Chiari syndrome. All 8 patients were female aged 20-75 yrs (median 29 yrs) ; 7 patients were native from th~ [lediterranean areas. An associated condition was documented in all patients : polycytheml. vera (3 cases), essential thrombocythemia (I case), agnogenic myeloid metaplasia (1 case), latent myeloproliferative disorders defined by the spontaneous formation of erythroid colonies in culture of bone marrow progenitors on erythropoietin-poor media (2 cases), immunoallergic vasculitis (i case). A short-length stenosis (6 cases) or a membrane (2 cases) involving one hepatic vein ostiu: or its vicinity was documented by ultrasounds and angiography (6 cases), necropsy (I case). or ultrasounds alone (i case). The other hepatic veins could not be cannulated (6/6), coul, not be demonstrated by ultrasounds (6/8), or appeared as an hyperechoic line (2/8) corresponding to a fibrous cord due to a concentric, non inflammatory, intimal thickening (l necropsy case). Recent thrombosis superimposed on ostial stenosis was documented in 2 cases. Percutaneous angioplasty failed fn a patient subsequently cured by surgical angioplasty. In conclusion, primary myeloproliferative disorders are the main cause of hepatic vein webs and short-length stenoses. Young Mediterranean women seems to be particularly affected. Platelet dysfunction and anatomical factors may concur to the formation of such lesions.
$188
Ullrich,D+;Grotefendt,M;Sieq,A;Bircher,J and W.Schr6ter + Departments of Clinical Pharmacology and Pediatrics+; University of G6ttingen;FRG. In patients with homozygous ~-thalassemia (thal) hyperbilirubinemia has been observed, even when erythropoesis had been suppressed by a high-frequency transfusion regimen and biochemical evidences for hepatic diseases were absent. It appeared pertinent therefore to further investigate the origin of this hyperbilirubinemia. Methods: 15 patients (6 males, 9 females, aged 1-23 y) with homozygous 5-±hal and 1 patient (male) with sicklecell-thal were studied: hemoglobin was 9.7±2.0 g/dl (mean ± ISD); haptoglobin 0.79±0.72 ng/ml; GPT 43±34 U/I; serum ferritin 2057±1225 ng/ml and density on CT-scan of the liver was 86.6±13.5 Houndsfield U (n:<80). Patients had neither signs for hepatitis B nor evidence for cirrhosis. 14 healthy infants (5 males, 9 females; 3-18 years) and 26 adults (14 males, 12 females, 19-63 years) served as controls. Total bilirubin was measured by diazo-method, bilirubin-conjugates were determined by alkaline methanolysis followed by thin-layer-chromatography. Results: In patients with ±hal total bilirubin was 24.1±22.6 pmol/l compared to 6.9±4.2 (p
HEPATIC VEIN WEBS AND SHORT-LENGTH STENOSES IN PATIENTS WITH PRI~MRY ['~ELOPROLIFERATIVE DISORDERS D. Valla, Y. Menu, M. Morcelet, G. Molas, J. Bel~hiti, H6pital Beaujon, 92118 Clichy, France
B. Rueff, and JP. Benhamou..
In some patients with Budd-Chiari syndrome, hepatic venous outflow block is caused by fibrous, short-length stenoses or webs involving the hepatic vein ostia or their vicinity. Such lesions have been variously attributed to a congenital, inflammatory, or thrombotic origin. We report the cases of 8 patients with such lesions among 56 consecutive patients with Budd-Chiari syndrome. All 8 patients were female aged 20-75 yrs (median 29 yrs) ; 7 patients were native from th~ [lediterranean areas. An associated condition was documented in all patients : polycytheml. vera (3 cases), essential thrombocythemia (I case), agnogenic myeloid metaplasia (1 case), latent myeloproliferative disorders defined by the spontaneous formation of erythroid colonies in culture of bone marrow progenitors on erythropoietin-poor media (2 cases), immunoallergic vasculitis (i case). A short-length stenosis (6 cases) or a membrane (2 cases) involving one hepatic vein ostiu: or its vicinity was documented by ultrasounds and angiography (6 cases), necropsy (I case). or ultrasounds alone (i case). The other hepatic veins could not be cannulated (6/6), coul, not be demonstrated by ultrasounds (6/8), or appeared as an hyperechoic line (2/8) corresponding to a fibrous cord due to a concentric, non inflammatory, intimal thickening (l necropsy case). Recent thrombosis superimposed on ostial stenosis was documented in 2 cases. Percutaneous angioplasty failed fn a patient subsequently cured by surgical angioplasty. In conclusion, primary myeloproliferative disorders are the main cause of hepatic vein webs and short-length stenoses. Young Mediterranean women seems to be particularly affected. Platelet dysfunction and anatomical factors may concur to the formation of such lesions.
$188