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Více než 10 let trvající kompletní remise monoklonální gamapatie nejistého významu a vymizení nefrotického syndromu vzniklého na podkladě light chain deposition disease po léčbě vinkristinem, adriamycinem a vysokými dávkami dexametazonu (VAD)
Title in English | More than 10 years of complete remission of MGUS and cessation of light chain deposition disease-associated nephrotic syndrome following treatment with vincristine, adriamycin and high-dose dexamethasone (VAD) |
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Authors | |
Year of publication | 2010 |
Type | Article in Periodical |
Magazine / Source | Vnitřní lékařství |
MU Faculty or unit | |
Citation | |
Field | Oncology and hematology |
Keywords | light chain deposition disease; monoclonal gammopathy of undetermined significance (MGUS); multiple myeloma; renal insuficiency; nephrotic syndrome; AL- amyloidosis |
Description | Light chain deposits in the form of amorphous material (light chain deposition disease) damage most frequently kidneys and, less frequently, they affect other organs. The incidence of light chain deposition disease is much lower than that of AL-amyloidosis. Symmetrical swelling of both legs, swelling of the eye lids, erythrocyturia and nephrotic proteinuria were the first signs of light chain deposition disease in our patient. The disease was diagnosed from kidney biopsy performed at the stage of advanced nephrotic syndrome with reduced filtration. The bone marrow aspirate contained 0.8% of plasma cells, serum contained monoclonal immunoglobulin IgG-kappa and urine contained free kappa chains. Blood count was normal and no osteolytic changes to the skeleton were identified. The patient was, therefore, diagnosed with monoclonal gammopathy of undetermined significance (MGUS) and was treated with 10 cycles ofchemotherapy consisting of vincristine, adriamycin and high/dose dexamethasone (VAD). |
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