Primary myelofibrosis in a patient who developed primary biliary cirrhosis, autoimmune hemolytic anemia and fibrillary glomerulonephritis
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We report a patient with primary myelofibrosis (PMF) who developed primary biliary cirrhosis, autoimmune hemolytic anemia, and fibrillary glomerulonephritis (FGN).
A 67-year-old woman was referred to our institution in November 1999 because of asthenia and nocturnal sweats associated to PMF, which was diagnosed in September 1996. In June 1998, high liver enzymes, circulating antimitochondrial antibodies, and a liver biopsy disclosed primary biliary cirrhosis. She had a history of arthralgia, positive latex test, xerosthalmia, and xerosthomia.
On admission, she presented with hepatosplenomegaly, moderate thrombocytosis, mild anemia, elevated leukocytes and 6% circulating blasts. Leukocyte alkaline phosphatase score was very low, karyotype was normal, no bcr-abl translocation was detected, and high lactic dehydrogenase levels were found. Bone marrow (BM) aspiration yielded a dry tap and BM biopsy disclosed megakaryocyte and myeloid hyperplasia, diffuse increase in reticulin...
KeywordsPrimary Biliary Cirrhosis Autoimmune Hemolytic Anemia JAK2V617F Mutation Osteosclerosis Primary Myelofibrosis