Member area login
You don't have or don't remember the password!
Click Here
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

--------------------

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



BC: Articoli scritti da R. Albertini

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
 
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
 
Un caso di gammopatia monoclonale di significato renale
A case of monoclonal gammopathy of renal significance
<p>Monoclonal gammopathy of renal significance (MGRS) is a condition defined by the presence of a small-B cell clone causing a renal disease trough deposition in renal tissues of the monoclonal component (MC) secreted by the B cells. Since MGRS is associated with several types of renal diseases, characterization of renal damage caused by protein deposition is important to define the correct diagnosis as well as the identification of the MC. Adult Fanconi Syndrome (FS) is characterized by the presence of a MC and damage in the proximal tubule with impaired small molecules transport. We report the case of a 32 years old man with moderate kidney failure, normoglycemic glycosuria and hypouricemia. Further investigations revealed hypophosphoremia and phosphaturia; an IgG&kappa; MC was detected by immunofixation. The kidney biopsy confirmed FS suspect. This case underlines that the results of the biochemical analysis carried on for the diagnosis of FS, need to be confirmed by histopathologic analysis.</p>
Biochimica Clinica ; 44(2) E08-E10
Casi Clinici - Case Report
 
Valutazione della risposta alla terapia in un paziente con amiloidosi AL e basse concentrazioni della catena leggera libera monoclonale
Evaluation of response to treatment in a patient with light chain amyloidosis and low free light chain burden
<p>Evaluation of response to treatment in a patient with light chain amyloidosis and low free light chain burden. In patients with light chain (AL) amyloidosis, reduction of amyloidogenic circulating free light chain (FLC) concentration translates in improvement of organ dysfunction and is associated with an increase in overall survival. Validated criteria for hematologic response to therapy are based on FLC quantification. However, patients with a difference between involved and uninvolved FLC (dFLC) &lt;50 mg/L are not evaluable for hematologic response. Here we report the case of a 69 year old man with AL (&lambda;) amyloidosis with renal involvement, presenting a low-FLC burden (dFLC 41 mg/L) at diagnosis. After two lines of treatment, a profound reduction of amyloidogenic FLC (dFLC 0 mg/L) was associated with an improvement of organ dysfunction. This case emphasizes the role of FLC assessment in the monitoring also of patients with a low-dFLC burden.</p><p>&nbsp;</p>
Biochimica Clinica ; 43(1) e4-e6
Casi Clinici - Case Report
 
La misura delle catene leggere libere indentifica la ricaduta di malattia e orienta per una rivalutazione della tipizzazione dell’amiloide in una paziente con amiloidosi AL
Free light measurement identifies relapse and prompts to reconsider amyloid typing in a patient with AL amyloidosis
<p>The detection and quantification of amyloidogenic light-chains (LC) is necessary for diagnosis and evaluation of response in AL amyloidosis. A 69 years old woman was initially diagnosed, in another center, with AL-<span style="font-family:calibri,sans-serif; font-size:11.0pt">&lambda;</span> amyloidosis with renal and soft tissue involvement in December 2001. After 4 cycles of therapy with melphalan and dexamethasone serum and urine immunofixation were negative and, after cycle 6, complete remission was confirmed. Free light chain (FLC) ratio was normal until June 2006, when proteinuria increased, and an elevated k-FLC concentration with abnormal k/<span style="font-family:calibri,sans-serif; font-size:14.6667px">&lambda;</span>-ratio was documented. We repeated the abdominal fat aspirate for amyloid typing by immune-electron microscopy that revealed k-LC deposits. The diagnosis was AL-k. A relapse was documented and the patient was started on bortezomib and dexamethasone therapy. After 8 cycles, complete remission was obtained. In this case, FLC allowed the identification of the amyloidogenic-LC, enabling the detection of relapse.</p>
Biochimica Clinica ; 42(2) e15-e17
Casi clinici - Case report
 
Ruolo del saggio Hevylite® nella diagnosi e nel monitoraggio delle gammopatie monoclonali
Role of Hevylite® assay in the diagnosis and monitoring of monoclonal gammopathies
<p>Clinical tests for detection and characterization of monoclonal immunoglobulins include serum and urine protein electrophoresis, and serum and urine immunofixation. The quantification of monoclonal components provides a surrogate for monitoring the size of malignant cell population in patients affected by plasma cell dyscrasia. As complementary test, immunoglobulin quantification is useful in patients with high concentrations of monoclonal IgG and in patients with monoclonal IgA whose electrophoretic migration is in the -fraction. Serum free light chain / ratio and the concentration of free light chains can also be used in different conditions. To overcome the limitations of traditional methods, e.g., for the quantification of monoclonal components that are indistinguishable from other proteins at electrophoresis, a new nephelometric immunoassay, called Hevylite assay (HLC), was developed. HLC separately measures in pairs light chain types of each intact immunoglobulin class, generating ratios of monoclonal immunoglobulin/uninvolved polyclonal immunoglobulin concentrations. Studies have shown that HLC and immunofixation are complementary methods. In this review, we summarize the role of HLC in the identification of clonality, prediction of prognosis in patients with multiple myeloma and in the evaluation of response to treatment. HLC ratio may also reveal immunoparesis and serve as a new marker for validating remission depth and relapse probability.</p>
Biochimica Clinica ; 40(4) 302-306
Rassegne - Reviews
 
Identificazione di danno renale reversibile e di precoce risposta alla chemioterapia in pazienti con amiloidosi AL
Identification of reversible renal damage and early response to chemotherapy in AL amyloidosis
<p>The kidney&nbsp;is involved in 70% of patients with immunoglobulin light-chain (AL) amyloidosis, but little is known on progression or&nbsp;reversibility of renal involvement. Furthermore, criteria for renal response have never been validated. We designed a&nbsp;staging system for renal damage and identified criteria for renal response and progression in a population of 732&nbsp;newly diagnosed patients with AL amyloidosis. The population was composed of 461 patients from Pavia (testing&nbsp;cohort) and 271 subjects from Heidelberg (validation cohort). Baseline proteinuria &gt;5 g/24 h and estimated glomerular&nbsp;filtration rate (eGFR) &lt;50 mL/min/1.73 m<sup>2</sup> were independently associated with poorer renal survival and discriminated&nbsp;between 3 stages (with none, one or two markers above the cut-off) with significant different renal survival. At 6-month&nbsp;follow-up, a &ge;25% eGFR decrease predicted poor renal survival in both cohorts and was adopted as criterion for renal&nbsp;progression. A decrease in proteinuria &ge;30% or below the cut-off of 0.5 g/24 h in absence of renal progression were&nbsp;the criteria for renal response, being associated with longer renal survival in the testing and validation cohorts. These&nbsp;endpoints can be used as validated response criteria in renal AL amyloidosis, allowing early assessment of treatment&nbsp;efficacy.</p>
Biochimica Clinica ; 40(1) 21-27
Contributi scientifici - Scientific Papers
 
Adrenomedullina e peptidi correlati: dalla fisiologia alla diagnostica
Adrenomedullin and related peptides: from physiology to diagnostics
<p>Adrenomedullin (ADM) and related peptides have important physiologic effects on cardiovascular system, including a potent and powerful hypotensive activity caused by dilatation of resistance vessels. Other roles are played in endocrine and nervous systems. The plasma concentrations of ADM are increased in several cardiac diseases, raising the possibility of a role as a biomarker of heart failure. The actions of ADM are generally protective and beneficial to organs and tissues (e.g., vasodilation, natriuresis and anti-inflammation) and suggest that increased ADM expression or activity could act as a compensatory response to end-organ injury. The investigations on the possible applications of ADM in clinical studies have increased since the introduction of an assay measuring mid-regional proadrenomedullin (MR-proADM), a peptide derived from proadrenomedullin characterized by a high stability in plasma. Recent studies suggest that MRproADM could be a marker of adverse prognostic effects after myocardial infarction or during acute cardiac dyspnea. Recently, our group showed that MR-proADM is a powerful prognostic marker in AL (light-chain) amyloidosis, which may not only reflect cardiac dysfunction, but also widespread systemic disease, and can be combined with cardiac troponin for detecting patients at risk of early death. However, further studies are required in order to recommend the assay of MR-proADM in the clinical laboratory; in particular, there is a lack of information about: 1) kinetics of MRproADM release after acute events and 2) the specific contribution given by MR-proADM assay in addition to the wellestablished determination of natriuretic peptides.</p>
Biochimica Clinica ; 37(2) 086-090
Rassegne - Reviews
 
La misura delle catene leggere libere delle immunoglobuline nel siero: aspetti analitici e clinici
Determination of free light chains in serum: analytical and clinical aspects
Biochimica Clinica ; 34(1) 26
RASSEGNE - RASSEGNE