A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent.
Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis. CONCLUSION. Portal hypertension is a complication of systemic mastocytosis that can lead to death. Treatment with interferon alpha might be effective.
Service de Pédiatrie, Hôpital Huriez, Lille, France.
H S
HS
Fonga-Djimi
F
F
Gottrand
M
M
Bonnevalle
J P
JP
Farriaux
D000293:Adolescent; D003594:Cytoplasmic Granules / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000473:pathology*; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000473:pathology; D008407:Mast Cells / Q000473:pathology; D011169:Portal Vein / Q000473:pathology; D013154:Spleen / Q000473:pathology; D014582:Urticaria Pigmentosa / Q000473:pathology*