Aggressive systemic mastocytosis mimicking sclerosing cholangitis.
A 43 year-old woman presented with fever, abdominal pain, epato-splenomegaly, ascites, cholestasis, anemia, thrombocytopenia and previous diagnosis of sclerosing cholangitis based on liver biopsy and endoscopic retrograde cholangiopancreatography(ERCP). The bone marrow biopsy and the revision of liver biopsy using antitryptase stain diagnosed systemic mastocytosis. Because of the aggressive course of the disease the patient was treated with an acute myeloid leukaemia chemotherapy regimen without success.
Marbello
Laura
L
Department of Oncology and Hematology, Niguarda Ca' Granda Hospital, Milan, Italy. ematologia@ospedaleniguarda.it <ematologia@ospedaleniguarda.it>
Anghilieri
Michela
M
Nosari
Annamaria
A
Minola
Ernesto
E
Cairoli
Roberto
R
Ricci
Francesca
F
Morra
Enrica
E
D015746:Abdominal Pain / Q000209:etiology; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D019943:Amino Acid Substitution; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology; D006502:Budd-Chiari Syndrome / Q000209:etiology; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis*; D002999:Clone Cells / Q000473:pathology; D003561:Cytarabine / Q000008:administration & dosage; D003937:Diagnosis, Differential; D003967:Diarrhea / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D005483:Flushing / Q000209:etiology; D006634:Histamine H1 Antagonists / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D008099:Liver / Q000473:pathology; D008223:Lymphoma / Q000175:diagnosis; D008407:Mast Cells / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D020125:Mutation, Missense; D017354:Point Mutation; D011241:Prednisone / Q000008:administration & dosage; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D012008:Recurrence
D000305:Adrenal Cortex Hormones; D006634:Histamine H1 Antagonists; D003561:Cytarabine; D019009:Proto-Oncogene Proteins c-kit; D011241:Prednisone; D015255:Idarubicin