Aggressive systemic mastocytosis mimicking sclerosing cholangitis.
نویسندگان
چکیده
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.
منابع مشابه
Systemic Mastocytosis Causing Refractory Pruritus in a Liver Disease Patient
Systemic mastocytosis (SM) results from clonal, neoplastic proliferation of abnormal mast cells. Patients become susceptible to itching, urticaria, and anaphylactic shock, which occurs due to histamine release from mast cells. SM may coexist alongside other systemic diseases, thus confounding the overall clinical presentation. We discuss a 23-year-old woman with refractory pruritus, which was i...
متن کاملPReS-FINAL-2080: Overlap syndrome between primary sclerosing cholangitis and autoimmune hepatitis associated with ulcerative colitis - a case of unusual presentation in a patient with systemic juvenile idiopathic arthritis
Introduction Systemic juvenile idiopathic arthritis (systemic-JIA) is characterized by the presence of arthritis associated with systemic manifestations and an important inflammatory response, being always a clinical diagnosis of exclusion. The association of systemic-JIA with autoimmune diseases is uncommon. The authors found only one description of systemic-JIA associated with autoimmune hepa...
متن کاملSarcoidosis mimicking primary sclerosing cholangitis requiring liver transplantation.
Sarcoidosis is a systemic granulomatous disease of unknown etiology. The association of the cholestatic pattern usually seen in sarcoidosis, with biliary duct changes resembling primary sclerosing cholangitis (PSC) is rare. Liver transplantation permits the histological evaluation of the complete explanted liver, making the diagnosis more reliable. In conclusion we present our experience with t...
متن کاملImmunoglobulin G4-associated sclerosing cholangitis mimicking cholangiocarcinoma.
Immunoglobulin G4-related lymphoplasmacytic sclerosing disease is an emerging disease. Recently, it has been shown to be responsible for autoimmune pancreatitis-induced strictures of the bile duct mimicking cholangiocarcinoma. Making a diagnosis of immunoglobulin G4-associated sclerosing cholangitis requires a high index of suspicion. The differential diagnoses include primary sclerosing cholan...
متن کاملA case of sclerosing cholangitis without pancreatic involvement thought to be associated with autoimmunity.
Sclerosing cholangitis (SC) is one of the lesions frequently seen in IgG4-related systemic diseases, causing biliary stricture and mimicking bile duct carcinoma and primary sclerosing cholangitis (PSC). Although it often accompanies autoimmune pancreatitis (AIP), autoimmune-related SC without a pancreatic lesion is very rare. A 79-year-old woman was referred to our institution with suspected di...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Haematologica
دوره 89 9 شماره
صفحات -
تاریخ انتشار 2004