[Fatal bleeding complications caused by Evans syndrome (autoimmune thrombocytopenia and hemolytic anemia) and type II autoimmune hepatitis in a 56-year-old patient].
BACKGROUND
Autoimmune hepatitis is a rare form of hepatitis of nonviral origin. Two main subentities have been described. The classical lupoid hepatitis (type I) is characterized by hypergammaglobulinemia and the presence of lupus erythematosus cells due to antinuclear antibodies. Autoimmune hepatitis type II, which is associated with antiliver/kidney microsomal antibodies type 1 (LKM 1) shows a more aggressive clinical course than autoimmune hepatitis type I and is frequently (41% of cases) associated with other immunologic diseases.


METHODS
In the present study we report a case of autoimmune hepatitis Type II, associated with autoimmune thrombocytopenia and hemolytic anemia, in a 56-year-old patient. The patient's death was caused by a fatal association of a failing coagulation system due to liver dysfunction and a severe autoimmune thrombocytopenia. The aggressive course of the thrombocytopenia even after splenectomy demonstrated that the splenic enlargement due to the portal hypertension was only a minor factor in the destruction of the thrombocytes. Interestingly, some findings of this case such as the advanced age, the presence of anti-smooth muscle antibodies and HLA-DR4 are usually associated with autoimmune hepatitis type I.


CONCLUSIONS
The findings of this case indicate that concomitant autoimmune diseases can worsen the prognosis of autoimmune hepatitis. Prednisolone and azathioprine might not be sufficient to treat aggressive forms of autoimmune hepatitis. Immunosuppressive regimens administered to recipients after organ transplantation might be used as a therapy of autoimmune hepatitis in multicenter clinical trials.
Zugmaier
Gerhard
G
Abteilung Hämatologie/Onkologie, Klinikum der Philipps-Universität Marburg. zugmaier@mailer.uni-marburg.de
Jäger
Robert
R
Neubauer
Andreas
A
Mennel
Hans-Dieter
HD
Knabbe
Cornelius
C
Dienes
Hans Peter
HP
D000744:Anemia, Hemolytic, Autoimmune / Q000473:pathology*; D001921:Brain / Q000473:pathology; D002543:Cerebral Hemorrhage / Q000473:pathology*; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D019693:Hepatitis, Autoimmune / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D011379:Prognosis; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000473:pathology*