Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Plasma cell leukaemia is a rare variant of multiple myeloma (2-3%), with an aggressive disease with short survival. It is defined as circulating peripheral blood plasma cells exceeding 2.000/ul and 20% of peripheral blood white cells. We present the case of 32-years-old man with left chest-wall pain, hepatomegaly, righ cervical adenopathy, 8800 leukocytes/ul with 33% of lymphocytes and 40% of plasma cell, 10.8 g/dl of proteins and a monoclonal hypergammaglobulinemia. Bone marrow biopsy showed 40-50 % of intramedullary plasma cell. There was a Ig G-kappa paraproteinemia.
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PMID:[Plasma cell leukaemia: a rare variant of multiple myeloma. A case report]. 1645 87

A 59-year-old man was referred to our hospital due to nephrotic syndrome with IgM paraproteinemia. Physical examination demonstrated marked hepatomegaly and anasarca. Serum M-protein was 0.94 g/dl and urinary analysis detected the presence of Bence Jones protein. Bone marrow plasma cell count was 11.2%. Histological examination demonstrated AL-type amyloid deposition in the liver, kidneys, bone marrow, stomach and rectum. These findings led to a diagnosis of IgM multiple myeloma with systemic amyloidosis. Although there was no apparent response to 2 courses of vincristine, doxorubicin and dexamethasone (VAD) regimen, subsequent treatment with bortezomib in combination with dexamethasone resulted in a rapid reduction in M protein to 0.49 g/dl, approximately half the pre-treatment level.
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PMID:[IgM-lambda multiple myeloma presenting with systemic amyloidosis]. 2006 79