Article Text

  1. R B Rocha1,
  2. N Silva1,
  3. S Martins1,
  4. T Pontes1,
  5. A Antunes1,
  6. H Antunes1
  1. 1Pediatrics Department, Hospital S. Marcos, Braga, Portugal


Autoimmune hepatitis is a chronic necroinflamatory liver disorder often associated with other autoimmune diseases. Association with idiopathic thrombocytopenic purpura is not frequent, especially in children.

We report a case of a 15-year-old boy who presented to our department with petechiae for 2 days and a 4-year history of a cervical posterior lymphadenopathy. Previous lab results 6 months previously showed elevated liver enzymes. He had a family history of psoriasis. At admission, physical examination was normal, except for purpura. Laboratory evaluation showed: platelet count, 6×109/L; aspartate aminotransferase 820 UI/L; alanine aminotransferase 1568 UI/L; normal gamma glutamyl transferase, alkaline phosphate, total bilirubin and unconjugated bilirubin levels; international normalized ratio (INR): 1.30; immunoglobulin G 1240 mg/dL (normal <1550 mg/dL); ferritin: 1230 ng/ml. Ceruplasmin, copper and alpha-1-antitrypsin levels were normal. He is heterozygous for the H63D of the HFE gene. Antinuclear, antimitochondrial, anti-smooth muscle and liver/kidney microssomal antibodies were negative. Blood smear, bone marrow aspirate and flux cytometry of peripheric blood cells was negative for neoplasic cells and pointed to peripheric destruction of platelets cells. Anti-platelet antibodies were positive. Liver biopsy, done after platelet account reached 50,000/uL, revealed an inflammatory lymphocytic infiltrate in portal areas, with many eosinophils and some areas of interface hepatitis and bridging fibrosis. He was successfully treated with prednisolone and azatioprine.

The aim of this presentation is to discuss an unusual association of autoimmune diseases: autoimmune hepatitis with immune thrombocytopenic purpura, in a male without hypergamaglobulinemia or autoantibodies at presentation.

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