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Editor-in-chief
Maria Stella Graziani

Deputy Director
Martina Zaninotto

Associate Editors
Ferruccio Ceriotti
Davide Giavarina
Bruna Lo Sasso
Giampaolo Merlini
Martina Montagnana
Andrea Mosca
Paola Pezzati
Rossella Tomaiuolo
Matteo Vidali

International Advisory Board Khosrow Adeli Canada
Sergio Bernardini Italy
Marcello Ciaccio Italy
Eleftherios Diamandis Canada
Philippe Gillery France
Kjell Grankvist Sweden
Hans Jacobs The Netherlands
Eric Kilpatrick UK
Magdalena Krintus Poland
Giuseppe Lippi Italy
Mario Plebani Italy
Sverre Sandberg Norway
Ana-Maria Simundic Croatia
Tommaso Trenti Italy
Cas Weykamp The Netherlands
Maria Willrich USA
Paul Yip Canada


Publisher
Biomedia srl
Via L. Temolo 4, 20126 Milano

Responsible Editor
Giuseppe Agosta

Editorial Secretary
Andrea di Bello
Biomedia srl
Via L. Temolo 4, 20126 Milano
Tel. 0245498282
email: biochimica.clinica@sibioc.it

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ISSN print: 0393 – 0564
ISSN digital: 0392- 7091



Articoli con TAG: catene leggere libere

Revisione e aggiornamento del documento di consenso SIBioC per la ricerca e quantificazione della proteina di Bence Jones
Update of the Italian Society of Clinical Biochemistry (SIBioC) Consensus document on the detection and quantification of the Bence Jones protein
<p>Bence Jones protein (BJP) refers to urine monoclonal free immunoglobulin light chains produced by the clonal expansion of a plasma cell in the bone marrow. BJP is strongly associated with systemic amyloidosis AL, light chain deposition disease, and multiple myeloma; less frequently, BJP may be recognized either in patients with monoclonal gammopathies of uncertain significance (MGUS) and with other plasma cell dyscrasias or in patients with malignant non-Hodgkin&#39;s lymphomas and chronic lymphocytic leukemia. This paper contains updated recommendations for the detection and the measurement of BJP in clinical practice from the Working Group &ldquo;Proteins&rdquo; of the Italian Society of Clinical Biochemistry (SIBioC), with specific indications for improving all the steps of the preanalytical, analytical, and postanalytical phases. The first morning void is the urine sample recommended for BJP detection, while 24-hours urine collection is preferred for BJP quantification. Native urine cannot be used for samples with low or very low content in urine total protein; in these cases, samples should be concentrated by using specific disposables, such as ultrafiltration membranes retaining proteins with molecular weight around 10 kDa. The required degree of concentration may vary according to sensitivity of the electrophoretic method utilized and the protein content of the sample. The detection of BJP may be performed directly by the recommended method agarose gel immunofixation (IFE) with specific polyvalent immunoglobulin antisera IgG-IgA-IgM, total  and  light chains; alternatively, an electrophoretic screening may be acceptable to rule out negative test results. However, positive test results should be confirmed by IFE. Tests based on immunometric methods can be used neither as screening test, nor for the BJP quantification; however, it could be useful for monitoring purposes, provided that the renal function of the patient is preserved. BJP measurement should be performed by the densitometric scanning of the electrophoretic peak corresponding to BJP, and results should be expressed as ratio of the BJP peak percentage to the urine total protein. Test results should be always integrated by standardized interpretative comments included in the laboratory reports.</p>
Biochimica Clinica ; 45(1) 075-086
Documenti SIBioC - SIBioC Documents
 
Catene leggere libere nella diagnostica liquorale della sclerosi multipla: possibile alternativa alla ricerca delle bande oligoclonali?
Free light chains in diagnosis of multiple sclerosis: an alternative to oligoclonal bands?
<p>Multiple sclerosis (MS) is one of the most common causes of neurological disability in young adults. MS presents heterogeneous clinical manifestations and both genetic and environmental factors are considered involved in the risk of developing the disease. The clinical diagnosis is rather complex reflecting the heterogeneity of the pathology. The diagnostic criteria, frequently modified over the years, require clinical symptoms, presence of typical lesions detected by magnetic resonance imaging and laboratory findings. The laboratory examination of the cerebrospinal fluid (CSF) allows an evaluation of inflammatory processes confined to the central nervous system reflecting the changes in the immunological pattern due to the progression of the pathology, playing thus an important role in the diagnosis and monitoring of MS. The detection of the oligoclonal bands (OCBs) is recognized as a &ldquo;gold standard&rdquo; for laboratory diagnosis of MS, although it suffers from methodological limitations. Indeed, OCBs assay is a manual multistep procedure, time-consuming that requires a subjective interpretation. In the last years, the measurement of the free light chains (FLC) in CSF appeared to assist in the diagnosis of MS. This procedure has been presented as a simpler and cheaper tool than the qualitative detection of OCBs. This article examines the current knowledge about the laboratory diagnostic of CSF, investigating both the validated method (OCBs) and the alternative biomarkers of immunoglobulins intrathecal synthesis, as the quantification of FLC in CSF.</p>
Biochimica Clinica ; 44(2) 157-167
Opinioni - Opinions
 
I marcatori di clonalità per la diagnosi e la valutazione della risposta alla terapia nell’amiloidosi da catene leggere: il ruolo del laboratorio
Clonal biomarkers for diagnosis and response to treatment assessment in light chain amyloidosis: the role of the laboratory
<p>Serum monoclonal components, Bence-Jones proteinuria (PBJ) and free light chains (FLC) are clonal biomarkers for diagnosis and response assessment in light chain (AL) amyloidosis. Two clinical reports are presented here toi llustrate the utility of these biomarkers. The first case is a patient with AL &kappa;appa renal amyloidosis. Serum and urine immunofixation were negative and the FLC ratio was abnormal. Immunoelectron microscopy on tissue biopsy was negative. Amyloid typing was achieved by mass spectrometry on fat pad aspirate. The second case is a patient with AL cardiac amyloidosis with PBJ lambdaand low concentration of amyloidogenic FLC (32 mg/L). Urine capillary electrophoresis was used to assess response to treatment. The progressive reduction of PBJ after treatment was accompanied by reduction of NT-proBNP and improvement of clinical conditions. Clonal biomarkers are irreplaceable tools in management of AL amyloidosis. There is a need for more sensitive techniques for identification of monoclonal FLC on serum and urine.</p>
Biochimica Clinica ; 44(2) E11-E15
Casi Clinici - Case Report
 
Una complicata valutazione della risposta alla terapia in un paziente con malattia da deposito di catene leggere libere
A complicated evaluation of the response to the therapy in a patient with light chain deposition disease
<p>Light chain deposition disease (LCDD) is characterized by tissue deposition, mostly in the kidney, of monoclonal immunoglobulin light chains (LCs), causing renal dysfunction and end-stage renal disease. The main goal of therapy is the reduction of LCs concentration, that can be obtained using chemotherapy approaches. We report the case of a 28-year-old man with LCDD (IgG&kappa; type) and underlying multiple myeloma who, after three ineffective lines of therapy, started a treatment with daratumumab, a monoclonal antibody (mAb, IgG1&kappa; type) drug, recently introduced for multiple myeloma treatment. The drug seemed effective but a IgG&kappa; spike remained visible at standard immunofixation. To discriminate the drug from the patient monoclonal component, immunofixation with Hydrashift system was used. This tool identified the visible IgG&kappa; as mAb drug and complete response was documented. This case showed the utility of new clinical assays for the evaluation of response to therapy in patients treated with mAb drugs.</p>
Biochimica Clinica ; 44(4) e30-e33
Casi Clinici - Case Report
 
Assenza di bande monoclonali liquorali e positività del rispettivo indice siero-liquor: una discrepanza da indagare attenatamente
Absence of liquoral monoclonal bands and positivity of the respective serum-liquor index: an unespected findings to be carefully investigated
<p>A 13 year-old child whit headache, nausea and repeated episodes of vomiting was hospitalized at the Major Hospital of Charity of Novara. A lumbar puncture and biochemical investigations on cerebrospinal fluid (CSF) including oligoclonal bands (OCB) screen were performed. Kappa and Lambda free light chains (kFLC and &lambda;FLC) and IgG concentrations were measured in serum and CSF by nephelometry. OCB were evaluated by isoelectric focusing (IEF) followed by immunofixation (IMF). A negative IgG index, no OCB IgG (type I pattern), positive kFLC index with increased levels of kFLC (0.14 mg/dL) and &lambda;FLC (0.14 mg/dl in CSF) were found. The FLC data suggested an intrathecal synthesis. A CSF IMF was then performed using anti-IgM peroxidase together with the IgM index using Reiber&rsquo;s hyperbolyc graphic. The presence of a monoclonal IgM band and 76% of intrathecal IgM synthesis was demonstrated. A subsequent molecular biology investigation was performed on CSF and revealed a clear positivity for enterovirus.</p>
Biochimica Clinica ; 44(4) e34-e36
Casi Clinici - Case Report