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E Pascali

Publications and source records attributed to E Pascali.

At least 19 recordsLinked to original sources

Clinical applications of immunoglobulin free light chain analysis.

The potential of immunoglobulin-free light chain detection and measurement in biological fluids, and particularly in urine, has not yet been fully explored in clinical medicine, because of differences in sensitivity and lack of standardization of both quantitative assays and qualitative analysis. The ability to identify monoclonal free light chain, i.e., Bence-Jones protein, reliably in urine is of great clinical interest even when it occurs only in such small amounts that they frequently remain undetected by the routine methods used in many laboratories. The standardized adoption of both qualitative and quantitative procedures with comparable sensitivity is suggested to allow precise characterization of the clonal nature, and therefore the clinical role, of any free light chain excess.

Bence Jones Protein

[Bence-Jones proteinuria in multiple sclerosis: a possible indicator of disease activity].

The protein patterns of the serum, cerebrospinal fluid and urine from 32 patients with definite multiple sclerosis and 30 control patients with neurological non-demyelinating disorders were simultaneously analyzed by means of high-resolution agarose gel electrophoresis combined with immunofixation. About one-third (31%) of the patients with multiple sclerosis were found to excrete monoclonal free light chains of immunoglobulins, i.e., Bence Jones proteins, in the urine without any concurrent immunoproliferative disease. Bence Jones proteinuria, at concentration generally not exceeding 0.2 g/24 hours, was demonstrated exclusively in multiple sclerosis patients in a phase of active disease (44% of the cases) or in chronic progressive disease (25% of the cases). The results of the study suggest that the presence of Bence Jones protein in the urine from multiple sclerosis patients may be utilized as an indicator of disease activity.

Adolescent

The clinical significance of pure Bence Jones proteinuria at low concentration.

The clinical significance of Bence Jones (BJ) proteinuria at low concentration (less than 0.2 g/24 hours) was investigated in 33 unselected patients who had no intact monoclonal immunoglobulin in their serum. The great majority (79%) of the patients were recognized as having malignant lymphoproliferative diseases, such as chronic lymphocytic leukemia (46%), hairy cell leukemia (6%), and non-Hodgkin's lymphoma (27%), whereas only two patients (6%) had multiple myeloma or systemic amyloidosis. Five patients (15%) had no evidence of definite malignant immunoproliferative disorders at the time of recognition of BJ proteinuria. Three of them, who were excreting steadily low amounts of BJ protein in their urine for 47, 61, and 73 months, respectively, without development of any B-lymphocytic malignancy, were classified as having a monoclonal gammopathy of undetermined significance. In the remaining two patients, BJ protein disappeared spontaneously 14 and 18 months, respectively, after its recognition. The study indicates that pure BJ proteinuria, even when occurring at low concentration, is most consistently associated with malignant proliferations of B-lymphocytic origin. However, the possibility should be considered that the clinical spectrum of the monoclonal gammopathy of the light chain type also includes benign and transient forms.

Adult

The clinical spectrum of pure Bence Jones proteinuria. A study of 66 patients.

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.

Aged

Bence Jones proteinuria in multiple sclerosis.

We report our findings of Bence Jones proteins (monoclonal free light chains of immunoglobulins) in concentrated urines of patients with multiple sclerosis, by using agarose electrophoresis and immunofixation. The lack of such findings in urines from healthy subjects and patients with other neurological disorders should stimulate further investigation.

Adolescent

Monoclonal Bence Jones proteinuria in chronic lymphocytic leukaemia.

A series of 52 consecutive patients with newly diagnosed chronic lymphocytic leukaemia (CLL) was investigated for the presence of serum and urine monoclonal immunoglobulins. The overall incidence of monoclonal gammopathy (MG) was 69%. Serum M components belonging to the IgG and IgM classes were found in 27% of cases with a concentration ranging from 1.7 to 40.3 g/l (mean 10 g/l). A monoclonal free light chain, i.e. Bence Jones protein (BJP), was documented in the urine of 65% of cases. In 42% of the 52 patients the urinary excretion of BJP represented the sole detectable monoclonal immunoglobulin abnormality. The occurrence of serum monoclonal immunoglobulins was not found to correlate with the stage or progression of the disease. Conversely, the isolated urinary excretion of BJP showed a relationship with the tumour load, as evaluated in terms of clinical enlargement of lymphoid areas. The presence of BJP alone was also a major distinctive feature of patients with more aggressive disease. These preliminary data would suggest that the isolated urinary excretion of BJP may represent a parameter of clinical significance in evaluating patients with CLL.

Adult

Serum and urine monoclonal immunoglobulins in malignant non-Hodgkin's lymphoma.

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 plasmacytoid 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.

Adult