This study was carried out in order to investigate a possible relationship between multiple myeloma and the occurrence of material exhibiting the properties of amyloid within renal tubules. Two groups of autopsied patients, with myelomatosis and benign monoclonal gammopathy were examined for the presence of amyloid deposits in renal and extra-renal sites. Urines were analysed for the presence and amount of Bence Jones protein and the pattern of the associated proteinuria was characterized. Renal tubular casts exhibiting the histochemical characteristics of immuno-amyloid were found exclusively in myeloma patients with Bence Jones proteinuria but without the renal lesions classically described as "myeloma kidney". This finding was independent of the occurrence of immuno-amyloid deposits in other renal and extra-renal sites, suggesting involvement of local factors in the pathogenesis of amyloid formation and deposition within renal tubular lumina. The results of present study suggest the conclusion that the presence of amyloid intratubular casts is to be regarded as a peculiar finding in myelomatosis.
62 consecutive patients with newly diagnosed malignant non-Hodgkin’s lymphoma (NHL) were investigated for the presence, type, and amount of serum and urine monoclonal immunoglobulin abnormalities. The overall incidence of monoclonal gammopathy (MG) was 81%. M components of the IgM and IgG classes were found in the serum of 52% of the patients. Their concentration was below 10 g/l in 54% of cases and above 20 g/l in 26% of cases. The highest incidence of serum M components (75%) was seen in plasmocytoid lymphocytic lymphoma (PLL) and the lowest (38%) in follicular center cell lymphoma. A monoclonal free light chain, i.e., Bence Jones protein (BJP), was documented in the urine of 61 % of cases with a daily excretion comprised between 0.01 and 9.24 g. The isolated urinary excretion of BJP was a major finding accounting for 36% of all MG found in association with NHL. It occurred in all histopathological subtypes with a frequency ranging from 17 % of PLL to 37 % of small lymphocytic lymphoma.
Sixty-six consecutive patients exhibiting isolated urinary excretion of monoclonal free light chains, i.e. Bence Jones protein (BJP), on screening investigation for serum and urine monoclonal immunoglobulins were studied in order to better define the spectrum of immunoproliferative disorders associated with such a protein abnormality. The typical plasma cell neoplasms accounted for only one third of the cases, multiple myeloma (MM) and systemic amyloidosis (AL) being diagnosed in 18% and 15% of the patients, respectively. Eighteen (27%) of the patients were recognized as having malignant nonHodgkin's lymphomas (NHL), 21 (32%) had chronic lymphocytic leukemia (CLL), and 2 (3%) had hairy cell leukemia (HCL). Three patients (5%) without apparent evidence of any malignant immunoproliferative disease were classified as having a monoclonal gammopathy of undetermined significance (MGUS). The greatest urinary concentrations of BJP were found in plasmacytic neoplasms, the daily excretion of MM patients being significantly higher than that of AL patients. Considerably lower BJP outputs were recorded in the other diseases, the lowest ones being associated with MGUS. NHL patients had a daily excretion four times higher as compared with that of CLL patients. The distribution of NHL by histologic type was: follicular center cell lymphomas (FCCL) 39%, small lymphocytic lymphoma (SLL) 33%, immunoblastic lymphoma (IBL) 17%, and plasmacytoid lymphocytic lymphoma (PLL) 11%. The highest BJP levels were found in PLL, and the lowest ones in FCCL. In CLL patients the amount of urinary BJP correlated significantly with the tumor load, as estimated by the number of enlarged lymphoid areas. The study suggests that detection and measurement of isolated urinary BJP may provide useful data for the clinical evaluation of a wide spectrum of immunoproliferative disorders.
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