scholarly journals Unusual thermal transition in the serum calorimetric profile of a patient diagnosed with multiple myeloma with secretion of monoclonal κ free light chains: a case report

2016 ◽  
Vol 2 (3) ◽  
pp. 416-426 ◽  
Author(s):  
Svetla Todinova ◽  
◽  
Sashka Krumova ◽  
Tonya Andreeva ◽  
Keranka Dimitrova ◽  
...  
2016 ◽  
Vol 12 (3) ◽  
pp. 1884-1888
Author(s):  
Elena De Santis ◽  
Serena Masi ◽  
Iole Cordone ◽  
Francesco Pisani ◽  
Cecilia Zuppi ◽  
...  

1979 ◽  
Vol 25 (1) ◽  
pp. 190-192 ◽  
Author(s):  
F R Dalal ◽  
S Winsten

Abstract A patient with massive proteinuria was discovered to have double light-chain disease. Immunological studies demonstrated monoclonal light chains of both the lambda and kappa type in urine. The light chains were separate and distinct and were not found to be a part of any of the whole molecule immunoglobulins such as IgG, IgM, IgA, IgD, or IgE. Uniqueness of the proteins was confirmed by column chromatography. Clinical studies showed that the patient had multiple myeloma.


2019 ◽  
Vol 12 (7) ◽  
pp. e229312
Author(s):  
Namrah Siddiq ◽  
Colin Bergstrom ◽  
Larry D Anderson ◽  
Srikanth Nagalla

Patients with multiple myeloma (MM) are at risk for acquired dysfibrinogenemia resulting in laboratory abnormalities and/or bleeding complications. We describe a 63-year-old man who presented with bleeding diathesis in the presence of a low fibrinogen activity level with a normal fibrinogen antigen level. Further studies revealed elevated levels of lambda free light chains, and he was diagnosed with MM. Despite initiating treatment with bortezomib/dexamethasone, he continued to have recurrent bleeds along with hypofibrinogenaemia, prompting a switch to carfilzomib/dexamethasone. The patient responded with improvement in bleeding symptoms, normalisation of fibrinogen activity and a decrease in serum free light chains.


2020 ◽  
Vol 8 (4) ◽  
pp. 617-624
Author(s):  
Uros Markovic ◽  
Valerio Leotta ◽  
Daniele Tibullo ◽  
Rachele Giubbolini ◽  
Alessandra Romano ◽  
...  

Nefrología ◽  
2018 ◽  
Vol 38 (3) ◽  
pp. 337-338
Author(s):  
Gioacchino Li Cavoli ◽  
Silvia Passanante ◽  
Onofrio Schillaci ◽  
Franca Servillo ◽  
Carmela Zagarrigo ◽  
...  

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4768-4768
Author(s):  
Alex G. Richter ◽  
Stephen Harding ◽  
Steve Rimmer ◽  
Guy Pratt ◽  
Aarnoud Huissoon ◽  
...  

Abstract Background: Heavy chain disease (HCD) is a rare lymphoproliferative disorder characterized by a monoclonal heavy chain (HC) unattached to a light chain (LC). IgGHCD or γHCD typically presents as a lymphoproliferative disorder with lymphadenopathy and hepatosplenomegaly. Myeloma has been described associated with γHCD but only with a second intact Ig paraprotein. This report describes a unique presentation of multiple myeloma with monoclonal free γ3HC and kappa free light chains. Case: A 34 year old gentleman presented with mild persistent neutropenia following two episodes of pneumonia, 18 months previously. He admitted to persistent night sweats but no other significant history. Baseline investigations revealed a mild anaemia, neutropenia and a large IgG paraprotein with no associated light chain. Bone marrow aspirate and trephine confirmed myeloma. The patient was treated with cyclophosphamide, thalidomide and dexamethasone and has had a very good partial remission. He is awaiting a sibling allogeneic peripheral blood stem cell transplant. Investigations and results: Serum Electrophoresis confirmed a large IgG paraprotein (23g/l) with no associated light chain in the serum and identified as γ3 subclass by radial immunodiffusion. Western blot showed the γ3HC was truncated with a large deletion. Markedly elevated free kappa (κ) LC (503.58 mg/l [3.30–19.4]) were found in the serum with gross skewing of the kappa/lambda ratio. Urine electrophoresis revealed separate γHC and κ LC paraproteins. Western blot of the fractionated urine protein demonstrated different sized κLC aggregates. Flow cytometry of the marrow aspirate revealed an unusual staining pattern; CD5,19,38,45+ve and CD20,22,23,34,56,138 –ve plasma cells. Cytoplasmic staining revealed 2 distinct populations of plasma cells, the first producing γ3HC and the second only free κLC. Cytogenetics and FISH analysis for 14q, p53 and c-myc abnormalities were normal. Discussion: This is the first description of a Biclonal Myeloma with separate plasma cell populations producing γ3HC and κLC paraproteins. The biclonality confirms the free HC occurs as a result of abnormal synthesis not cleavage. The clinical and immunological findings are clearly different to typical findings in both γ3HCD and Myeloma. HCD has an appalling prognosis and this case is likely to have been ‘smouldering’ for 18 months, evidenced by the 2 pneumonias and persistent night sweats. There is no lymphadenopathy or organomegaly associated with γ3HCD. The immunophenotype of the malignant plasma cells is unique. Other atypical features include frank proteinuria, with a HC in the urine, but normal renal function and no radiological or biochemical evidence of bone involvement. We propose that this unique biclonal myeloma has distinct immunological and clinical features.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2927-2927
Author(s):  
Efstathios Koulieris ◽  
Dimitrios Maltezas ◽  
Nikolaou Eytychia ◽  
Vassiliki Bartzis ◽  
Tatianna Tzenou ◽  
...  

Abstract Abstract 2927 Background and Aims: Multiple Myeloma (MM) is characterized by bone marrow (BM) plasma cell infiltration and the presence of serum/urine monoclonal immunoglobulin (Ig). The depth of response has been associated with longer PFS in MM causing subsequent prolonged survival. Recently novel M-based biomarker immunoassays have been developed (Freelite™, Hevylite™) and their significance in MM diagnosis and prognosis has been demonstrated.1,2 Furthermore serum Free Light Chains (sFLC) are used for better assessment of treatment response, thus patients are considered to achieve stringed Complete Response (sCR) by having CR criteria plus normal serum Free Light Chains Ratio (sFLCR) and absent clonal cells on BM.3 The significance of Hevylite™ on response has not been assessed so far. Patients in nCR or better do not automatically restore their ratio of intact monoclonal Ig/intact polyclonal Ig of the same class (Hevylite™ or HLCR). We therefore investigated the importance of sFLCR and HLCR normalisation at plateau on PFS, in a series of patients with intact Ig MM. Patients and Methods: 50 intact immunoglobulin MM patients were studied from diagnosis to last follow up. Immunofixation was IgG (26 -kappa and 12 –lamdba) and IgA (6 –kappa and 6 -lambda). All patients were symptomatic at diagnosis. Sera samples (n=312) were analyzed for sFLC-kappa and sFLC-lambda with Freelite™ and sFLCR were calculated, and for IgGkappa, IgGlambda IgAkappa, IgAlambda with Hevylite™ and ratios IgGkappa/IgGlambda, IgGlambda/IgGkappa, IgAkappa,/IgAlambda and IgAlambda/IgAkappa (HLCRs) were calculated. sFLCRs and HLCRs values above the 95%-ile of normal individuals were considered abnormal. Statistical analysis was performed using SPSS ver 15.0. File data were reviewed. Results: At diagnosis sFLCR was abnormal in 86% of patients while HLCR was abnormal in all. All treatment lines were initiated according to standard criteria and median lines of therapy were 2 (range 1–11). Median follow up was 33 months (7–145). During patients' cumulative follow-up, 145 lines of therapy were studied and the subsequent responses were estimated. Thirty eight percent of responses were sCR, CR and nCR, 20% PR, 18% MR and 24% refractory and progressive disease. HLCR normalized in 44% of patients with sCR, CR and nCR. The depth of response correlated to PFS and patients in sCR, CR and nCR had longer PFS than the others (p<0.001). Serum FLCR and HLCR normal values at response were both strong parameters of increased PFS after treatment at any line (p=0.035 and p=0.046 respectively). Conclusion: Serum HLCR normalization at plateau reflects prolonged responses in intact Ig MM. Disclosures: Harding: Binding Site: Employment. Bradwell:The Binding Site: shareholder Other.


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