Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic...
1
HEPATIC VEIN THROMBOSIS IN PAROXYSMAL NOCTURNAL HEMOGLOBINURIA. A SPECTRUM FROM ASYMPTOMATIC VENO-OCCLUSIVE DISEASE TO FATAL BUDD-CHIARI SYNDROME D.Valla~ D.Dhumeaux, G.Babany, P.Hillon~ B.Rueff, J.P.Benhamou. HSpital Beaujon, Clichy, and H6pital Henri Mondor, Cr~teil, France In a series of 40 consecutive patients with hepatic vein obstruction, 5 (12%) were suffering from paroxysmal nocturnal hemoglobinuria (PNH). This prevalence is out of proportion to the prevalence of PNH in the general population (estimated to be 1•5/106 in France) and makes PNH the second cause of hepatic vein thrombosis, next to the primury myeloproliferative disorders. The clinicopathologica] features in these 5 patients and in the 26 well documented previously reported cases could be ascribed to 3 groups of increasing severity. In group I (6 patients), thrombosis was limited to the small-sized hepatic veins and the large-sized hepatic veins were patent: ascites was absent or transient; jaundice was absent; death was not related to the liver disease. In group II (10 patients), the large-sized hepatic veins were partially thrombosed: ascites was present in most of these patients; jaundice was uncommon; death was related to the liver disease in a third of them. In group Ill (15 patients), large-sized hepatic veins were completely thrombosed; ascites was present, massive, and usually intractable; jaundice was present in half of these patients; most of them died and death was related to the liver disease. We suggest that these 3 groups of patients represent, not different types, but successive stages, of hepatic venous obstruction. It is concluded that: (1) PNH, although a rare disorder, is a relatively common cause of hepatic vein obstruction; (2) hepatic vein obstruction complicating PNH might be confined primarily to small-sized hepatic veins (veno-occlusive disease) and extend secondarily to large-sized hepatic veins (Budd-Chiari syndrome); and (3) appropriate therapy at the stage of small-sized hepatic vein obstruction or at the early stage of large-sized hepatic vein obstruction might limit the extension of thrombosis and improve the prognosis of this severe complication of PNH. NATURAL HISTORYOF HBsAg + CHRONICHEPATITIS IN CHILDREN • A.Vegnente, P.Toscano, V.Nuzzo, L.Cimmino, R. lorio, L.Tarallo, A.M.Memoli*, S.Guida, C.Fusco. Inst. of Pediatrics, 2nd School of Medicine, Univ. of Naples, Naples. *Inst. of General Pathology, 2nd Sch. of Medicine, Univ. of Naples, Naples. We have followed up clinical conditions, growth and liver function (LFT s) (AST,AIb,TP,~GT and Ig) of 85 children (59 males, mean age 5 years, 6 months) affected by HBsAg +, HBeAg + Chronic Hepatitis (CH) for a period between I and 5 years• Liver biopsy was performed in 30 patients, revealing a medium activity CH. None of the patients ever received any immunosuppressive treatment. All of them maintained good health and normal height and weight gain. LFT s were also normal, except for AST,65 of the children(47 males,mean age 8 years,t0 months)were per- sistently HBeAg+; of them, 25 show normal (N)AST, 25 have 2N, 13 4N, 2 8N, 8 children (4 ma- les, mean age 9 years, 4 months) have been HBeAg- and HBeAb-for 13.8 months; I has normal AST, 5 2N, 2 4N; 12 children (8 males, mean age 9 years, 6 months) seroconverted to HBeAb after a mean of 28.2 months; one of them always had normal AST, one persistently showed ele- vated AST, 10 normalized their AST: 8 before the seroconversion, 2 after 8 and 21 months respectively. In one child the appearence of HBeAb was simultaneous with the disappearance of HBeAgi in 11 children there was an "e window" lasting 8.1 months. In conclusion the HBsAg + CH in our 85 patients was symptomsless and without grossly alteration of LFTs and also the seroconversion to HBeAb didn't relate to significant alterations of the biochemical pattern. Is the HBsAg + CH in children less severe than in adults because of a peculiar age-related immunological response or because it is just the beginning of a longlasting disease? S171
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Transcript of Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic...
HEPATIC VEIN THROMBOSIS IN PAROXYSMAL NOCTURNAL HEMOGLOBINURIA. A SPECTRUM FROM ASYMPTOMATIC VENO-OCCLUSIVE DISEASE TO FATAL BUDD-CHIARI SYNDROME
D.Valla~ D.Dhumeaux, G.Babany, P.Hillon~ B.Rueff, J.P.Benhamou. HSpital Beaujon, Clichy, and H6pital Henri Mondor, Cr~teil, France
In a series of 40 consecutive patients with hepatic vein obstruction, 5 (12%) were suffering from paroxysmal nocturnal hemoglobinuria (PNH). This prevalence is out of proportion to the prevalence of PNH in the general population (estimated to be 1•5/106 in France) and makes PNH the second cause of hepatic vein thrombosis, next to the primury myeloproliferative disorders.
The clinicopathologica] features in these 5 patients and in the 26 well documented previously reported cases could be ascribed to 3 groups of increasing severity. In group I (6 patients), thrombosis was limited to the small-sized hepatic veins and the large-sized hepatic veins were patent: ascites was absent or transient; jaundice was absent; death was not related to the liver disease. In group II (10 patients), the large-sized hepatic veins were partially thrombosed: ascites was present in most of these patients; jaundice was uncommon; death was related to the liver disease in a third of them. In group Ill (15 patients), large-sized hepatic veins were completely thrombosed; ascites was present, massive, and usually intractable; jaundice was present in half of these patients; most of them died and death was related to the liver disease. We suggest that these 3 groups of patients represent, not different types, but successive stages, of hepatic venous obstruction.
It is concluded that: (1) PNH, although a rare disorder, is a relatively common cause of hepatic vein obstruction; (2) hepatic vein obstruction complicating PNH might be confined primarily to small-sized hepatic veins (veno-occlusive disease) and extend secondarily to large-sized hepatic veins (Budd-Chiari syndrome); and (3) appropriate therapy at the stage of small-sized hepatic vein obstruction or at the early stage of large-sized hepatic vein obstruction might limit the extension of thrombosis and improve the prognosis of this severe complication of PNH.
NATURAL HISTORY OF HBsAg + CHRONIC HEPATITIS IN CHILDREN
• A.Vegnente, P.Toscano, V.Nuzzo, L.Cimmino, R. lor io, L.Tarallo, A.M.Memoli*, S.Guida, C.Fusco.
Inst. of Pediatrics, 2nd School of Medicine, Univ. of Naples, Naples. *Inst. of General Pathology, 2nd Sch. of Medicine, Univ. of Naples, Naples.
We have followed up cl inical conditions, growth and l i ver function (LFT s) (AST,AIb,TP,~GT and Ig) of 85 children (59 males, mean age 5 years, 6 months) affected by HBsAg +, HBeAg + Chronic Hepatitis (CH) for a period between I and 5 years• Liver biopsy was performed in 30 patients, revealing a medium act iv i ty CH. None of the patients ever received any immunosuppressive treatment. All of them maintained good health and normal height and weight gain. LFT s were also normal, except for AST,65 of the children(47 males,mean age 8 years,t0 months)were per- sistently HBeAg+; of them, 25 show normal (N)AST, 25 have 2N, 13 4N, 2 8N, 8 children (4 ma- les, mean age 9 years, 4 months) have been HBeAg- and HBeAb-for 13.8 months; I has normal AST, 5 2N, 2 4N; 12 children (8 males, mean age 9 years, 6 months) seroconverted to HBeAb after a mean of 28.2 months; one of them always had normal AST, one persistently showed ele- vated AST, 10 normalized their AST: 8 before the seroconversion, 2 after 8 and 21 months respectively. In one child the appearence of HBeAb was simultaneous with the disappearance of HBeAgi in 11 children there was an "e window" lasting 8.1 months. In conclusion the HBsAg + CH in our 85 patients was symptomsless and without grossly alteration of LFTs and also the seroconversion to HBeAb didn't relate to significant alterations of the biochemical pattern. Is the HBsAg + CH in children less severe than in adults because of a peculiar age-related immunological response or because i t is just the beginning of a longlasting disease?
S171
