doc26inder
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- The presence of a monoclonal light chain in the urine of a patient with nephrotic syndrome is almost always associated with what?( two conditions ) The presence of a monoclonal protein in the serum or in the urine in a patient with an unexplained nephrotic syndrome, congestive heart failure, sensorimotor peripheral neuropathy, carpal tunnel syndrome, or orthostatic hypotension is suggestive of what? How will you confirm your diagnosis ? How will u treat this case
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| nisha
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I guess that this question has something to do with amyloid?
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| ARJ
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DD of monoclonal light chain in the urine with presenting symptoms
1 Amyloidosis is sure the one of the diagnosis , Confirm the diagnosis with Rectal or Gingival Biopsy with Congo red+ Fluorescent Microscopy showing apple green fluorescence. There is no effective treatment death usually occurs within 1-3 years. Can excise the amyloid tumors
2 Waldenstrom Macroglobulinemia ? Light chain in 10 % of the cases. But not sure ??????????
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| doc26inder
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- The presence of a monoclonal light chain in the urine of a patient with nephrotic syndrome is almost always associated with primary amyloidosis (AL) or light chain deposition disease. The presence of a monoclonal protein in the serum or in the urine in a patient with an unexplained nephrotic syndrome, congestive heart failure, sensorimotor peripheral neuropathy, carpal tunnel syndrome, or orthostatic hypotension is suggestive of primary amyloidosis. Tissue must be obtained to make a diagnosis of primary amyloidosis. A subcutaneous fat aspirate and/or a positive bone marrow stain for amyloid is found in 90% of patients. If the clinician still suspects primary amyloidosis in a patient with a negative subcutaneous fat aspirate and bone marrow biopsy, he/she should obtain tissue from a clinically involved organ.
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Treatment for light chain deposition disease is the same as that for multiple myeloma. In this patient treatment must be aimed at reducing the mass of plasma cells that are producing the monoclonal kappa protein.
Options include the use of corticosteroids, alkylating agents or autologous peripheral blood stem cell transplant.
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