{"title":"Od monoklonalnej gammapatii o nieokreślonym znaczeniu (MGUS) do szpiczaka plazmocytowego i amyloidozy — opis przypadku","authors":"Michał Bator, S. Potoczek, L. Usnarska-Zubkiewicz","doi":"10.5603/HEM.2018.0040","DOIUrl":null,"url":null,"abstract":"We present a 69-year-old patient, in whom in March 2016 a monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. The disease was found during the diagnostics of thrombocytopenia (from 2009 the number of platelets varied between 90–140 G/l). In serum immunofixation assay the presence of monoclonal IgG kappa protein was confirmed. In July 2017, when the patient presented with nephrotic syndrome and acute renal failure, 14.5% of plasma cells were found in the bone marrow. Moreover, a biopsy of abdominal fat revealed the presence of amyloid composed of kappa light chains. The diagnosis of IgG kappa plasma cell myeloma (PCM) complicated with amyloidosis of the kidneys, heart, liver and spleen was established. The PCM progression complicated with amyloidosis was very aggressive. Despite the treatment with bortezomib and dexamethasone the patient died early. Sudden deterioration of health (e.g. in the form of acute renal failure) in a patient with MGUS may indicate not only the progression of MGUS to PCM, but also the development of amyloidosis, which is associated with turbulent disease and poor prognosis.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":"28 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2019-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hematologia","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.5603/HEM.2018.0040","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
Abstract
We present a 69-year-old patient, in whom in March 2016 a monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. The disease was found during the diagnostics of thrombocytopenia (from 2009 the number of platelets varied between 90–140 G/l). In serum immunofixation assay the presence of monoclonal IgG kappa protein was confirmed. In July 2017, when the patient presented with nephrotic syndrome and acute renal failure, 14.5% of plasma cells were found in the bone marrow. Moreover, a biopsy of abdominal fat revealed the presence of amyloid composed of kappa light chains. The diagnosis of IgG kappa plasma cell myeloma (PCM) complicated with amyloidosis of the kidneys, heart, liver and spleen was established. The PCM progression complicated with amyloidosis was very aggressive. Despite the treatment with bortezomib and dexamethasone the patient died early. Sudden deterioration of health (e.g. in the form of acute renal failure) in a patient with MGUS may indicate not only the progression of MGUS to PCM, but also the development of amyloidosis, which is associated with turbulent disease and poor prognosis.
期刊介绍:
Hematology is the quarterly under auspices of the Institute of Hematology and Transfusion Medicine. The journal is addressed to hematologists, oncologists and also internists. It contains the overview/review articles, case reports, essays, including reports from the scientific and educational conferences as well as test questions on hematology. Journal of the Institute of Hematology and Transfusiology.