A case of primary immunoglobulin light chain amyloidosis with a delayed appearance of Bence Jones protein in urine
| Autor: | Isao Shirato, Toshiyuki Yamada, Kyoichi Tashiro, Satoshi Horikoshi, Kenji Yamaji, Atsushi Kurusu, Miho Nishitani, Hisaki Rinno, Yasuhiko Tomino, Kunimi Maeda |
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| Rok vydání: | 2004 |
| Předmět: |
Male
Pathology medicine.medical_specialty Time Factors business.industry Amyloidosis General Medicine Middle Aged Immunoglobulin light chain medicine.disease Bence Jones protein Immunoglobulin Light-chain Amyloidosis Amyloid A Protein AA amyloidosis Nephrology medicine AL amyloidosis Humans Immunoglobulin Light Chains business Immunostaining Bence Jones Protein |
| Zdroj: | Nephrology (Carlton, Vic.). 9(3) |
| ISSN: | 1320-5358 |
| Popis: | We report here a case of a 58-year-old man who had nephrotic syndrome and immunoglobulin light chain (AL) amyloidosis. This patient underwent a renal biopsy to confirm the diagnosis. Treatment with permanganate before Congo red staining showed systemic secondary amyloidosis (AA) fibrils, which were sensitive to permanganate oxidation. Although this patient was initially diagnosed as having AA amyloidosis, he did not have any chronic inflammatory disease and/or malignancy. The level of amyloid A protein (7.9 microg/mL) in sera was within the normal range (0-8.0 microg/mL). Therefore, we performed an immunostaining of the precursor protein (amino terminus of constant region: kappa and lambda light chains, and AA protein) using duodenal biopsy specimens for a precise diagnosis. Immunostaining was positive for the amino terminus of constant region of the lambda light chain, and negative for the amino terminus of constant region of the kappa light chain and AA protein. No plasma cell proliferation in the bone marrow was observed. We finally diagnosed this patient as having primary AL amyloidosis. It appears that a pathological diagnosis must be performed by immunostaining the precursor proteins with the permanganate digestion technique in tissue of patients with amyloidosis. There were no abnormalities in serum and urine immunoelectrophoresis at the time of renal biopsy in this patient. During the follow-up period, after discharge, Bence Jones protein appeared in the urine, but not in the serum. It is necessary to observe patients with primary AL amyloidosis carefully to determine if they their condition will progress to multiple myeloma. |
| Databáze: | OpenAIRE |
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