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
Kjell Grankvist Sweden
Hans Jacobs The Netherlands
Eric Kilpatrick UK
Magdalena Krintus Poland
Giuseppe Lippi Italy
☩Howard Morris Australia
Mario Plebani Italy
Sverre Sandberg Norway
Ana-Maria Simundic Croatia
☩Jill Tate Australia
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
Arianna Lucini Paioni
Biomedia srl
Via L. Temolo 4, 20126 Milano
Tel. 0245498282
email: biochimica.clinica@sibioc.it



Area soci
Non possiedi o non ricordi la password!
Clicca qui

BC: Articoli scritti da P. Milani

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
 
Utilità del saggio Hevylite nella gestione clinica di una paziente affetta da amiloidosi AL con gammopatia biclonale
Usefulness of the Hevylite assay in the management of a patient with AL amyloidosis and biclonal gammopathy
<p>Patients with AL amyloidosis often have small monoclonal components (MCs) difficult to quantify by densitometry. IgA are the most problematic, due to anodic migration and possible masking under proteins migrating in &beta; zone. We evaluated the usefulness of the Hevylite assay (Binding Site, Birmingham UK), at diagnosis and during follow-up, in a patient with AL amyloidosis and biclonal gammopathy. At diagnosis serum immunofixation identified an IgG&lambda; and an IgA&lambda; band (the last one not reliably quantifiable in capillary electrophoresis). The &kappa; serum free light chain (FLC) concentration was 4.94 mg/L and &lambda; 26 mg/L (&kappa;/&lambda; ratio 0.19). The Hevylite test showed both IgG&lambda; and IgA&lambda; above the reference limits, with abnormal &kappa;/&lambda; ratios. After treatment, a 27% decrease in IgG&lambda; and a 56% decrease in IgA&lambda; concentration were documented by Hevylite, which was the only mean to quantify the monoclonal components in this patient.</p>
Biochimica Clinica ; 40(2) e12-e15
Casi clinici - Case report
 
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
 
Componenti monoclonali piccole ma dannose
Small but harmful monoclonal components
<p>A small B-cell clone can synthesize a toxic monoclonal protein that&nbsp;causes severe organ damage. Diseases caused by these toxic immunoglobulins range from AL amyloidosis, light&nbsp;chain deposition disease and cryoglobulinemia to the POEMS (Polineuropathy, Organomegaly, Endocrinopathy,&nbsp;Monoclonal protein and Skin changes) syndrome. The serum monoclonal components are, in these cases, usually&nbsp;small and difficult to be detected by the commercially available methods. However, an early detection and a correct&nbsp;characterization of the monoclonal component is essential, because misdiagnosis or a delay in diagnosis can prevent&nbsp;the proper treatment of the patient worsening his outcome. Here we present two cases of amyloidosis AL, where the&nbsp;correct diagnosis was facilitated by specific and sensitive techniques. This does not mean that every clinical&nbsp;laboratory should be competent in this field and use these methods routinely, but simply that people should be aware&nbsp;of the problem, so that these clinical conditions can be properly managed.</p>
Biochimica Clinica ; 37(5) 431-434
Casi clinici - Case Report
 
Un caso di gammopatia monoclonale risolto grazie ai biomarcatori cardiaci
A case of monoclonal gammopathy solved with cardiac biomarkers
Biochimica Clinica ; 35(3) 262
CASI CLINICI - CASI CLINICI