scholarly journals Role of light chain variable region in myeloma with light chain deposition disease: evidence from an experimental model

Blood ◽  
1995 ◽  
Vol 86 (10) ◽  
pp. 3655-3659 ◽  
Author(s):  
AA Khamlichi ◽  
A Rocca ◽  
G Touchard ◽  
P Aucouturier ◽  
JL Preud'homme ◽  
...  
Keyword(s):  
Experimental Model ◽  
Light Chain ◽  
Variable Region ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Kappa Chain ◽  
V Region ◽  
Light Chain Deposition

Light chain deposition disease (LCDD) results from a propensity of some human monoclonal L chains to form tissue deposits. We designed an experimental model for in vivo expression of human kappa L chain sequences in mice and compared a somatically mutated LCDD chain with a closely related control kappa chain, both encoded by the unique V kappa IV gene. Mice secreting the LCDD chain but not those producing the control chain showed deposits with a distribution similar to that observed in patients. These data show that discrete changes in V region sequences can play a major role in tissue deposition of human L chains.

scholarly journals Primary cystic lung light chain deposition disease: a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor

Blood ◽  
2008 ◽  
Vol 112 (5) ◽  
pp. 2004-2012 ◽  
Author(s):  
Magali Colombat ◽  
Hervé Mal ◽  
Christiane Copie-Bergman ◽  
Jacques Diebold ◽  
Diane Damotte ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
B Cell ◽  
Light Chain ◽  
Cell Clone ◽  
Variable Region ◽  
Amino Acid Identity ◽  
Light Chain Deposition Disease ◽  
Cystic Lung ◽  
Deposition Disease ◽  
Light Chain Deposition

Abstract We have recently described a new form of light chain deposition disease (LCDD) presenting as a severe cystic lung disorder requiring lung transplantation. There was no bone marrow plasma cell proliferation. Because of the absence of disease recurrence after bilateral lung transplantation and of serum-free light chain ratio normalization after the procedure, we hypothesized that monoclonal light chain synthesis occurred within the lung. The aim of this study was to look for the monoclonal B-cell component in 3 patients with cystic lung LCDD. Histologic examination of the explanted lungs showed diffuse nonamyloid κ light chain deposits associated with a mild lymphoid infiltrate composed of aggregates of small CD20+, CD5−, CD10− B lymphocytes reminiscent of bronchus-associated lymphoid tissue. Using polymerase chain reaction (PCR), we identified a dominant B-cell clone in the lung in the 3 studied patients. The clonal expansion of each patient shared an unmutated antigen receptor variable region sequence characterized by the use of IGHV4-34 and IGKV1 subgroups with heavy and light chain CDR3 sequences of more than 80% amino acid identity, a feature evocative of an antigen-driven process. Combined with clinical and biologic data, our results strongly argue for a new antigen-driven primary pulmonary lymphoproliferative disorder.

scholarly journals Primary structure of a variable region of the VI subgroup (ISE) in light chain deposition disease

2008 ◽  
Vol 91 (3) ◽  
pp. 506-509 ◽  
Author(s):  
A. ROCCA ◽  
A. A. KHAMLICHI ◽  
P. AUCOUTURIER ◽  
L.-H. NOËL ◽  
L. DENOROY ◽  
...  
Keyword(s):  
Light Chain ◽  
Primary Structure ◽  
Variable Region ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

Light Chain Deposition Disease Detected by Antisera to a Variable Region of the κ1 Light Chain Subgroup

Nephron ◽  
1998 ◽  
Vol 80 (2) ◽  
pp. 162-165 ◽  
Author(s):  
Shin-ichi Takeda ◽  
Eisuke Takazakura ◽  
Joji Haratake ◽  
Yoshinobu Hoshii
Keyword(s):  
Light Chain ◽  
Variable Region ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

Recovery from LCDD-associated Severe Liver Cholestasis: a Case Report and Literature Review

2016 ◽  
Vol 25 (1) ◽  
pp. 99-103 ◽  
Author(s):  
Benoit Brilland ◽  
Johnny Sayegh ◽  
Anne Croue ◽  
Frank Bridoux ◽  
Jean-François Subra ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Intrahepatic Cholestasis ◽  
Relevant Literature ◽  
Gamma Glutamyl Transferase ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Uncommon Presentation ◽  
Light Chain Deposition ◽  
Glutamyl Transferase

Light chain deposition disease (LCDD) is a rare multisystemic disorder associated with plasma cell proliferation. It mainly affects the kidney, but liver and heart involvement may occur, sometimes mimicking the picture of systemic amyloidosis. Liver disease in LCDD is usually asymptomatic and exceptionally manifests with severe cholestatic hepatitis. We report the case of a 66-year-old female with κ-LCDD and cast nephropathy in the setting of symptomatic multiple myeloma who, after a first cycle of bortezomib-dexamethasone chemotherapy, developed severe and rapidly worsening intrahepatic cholestasis secondary to liver κ-light chain deposition. Intrahepatic cholestasis was attributed to LCDD on the basis of the liver histology and exclusion of possible diagnoses. Chemotherapy was maintained and resulted in progressive resolution of cholestasis. We report here an uncommon presentation of LCDD, with prominent liver involvement that fully recovered with bortezomib-based chemotherapy, and briefly review the relevant literature. Abbreviations: AKI: Acute kidney injury; ALP: alkaline phosphatase; ALT: alanine aminotransferase; AST: aspartate aminotransferase; CMV: Cytomegalovirus; EBV: Epstein–Barr virus; GGT: gamma-glutamyl transferase; HSV: Herpes simplex virus; LC: light chain; LCDD: Light chain deposition disease; MIDD: Monoclonal immunoglobulin deposition disease; MM: Multiple myeloma.

scholarly journals Light Chain Deposition Disease Diagnosed Using Computed Tomography-Guided Kidney Biopsy

Cureus ◽  
2021 ◽  
Author(s):  
Yoshinosuke Shimamura ◽  
Yayoi Ogawa ◽  
Hideki Takizawa ◽  
Toshiaki Hayashi ◽  
Yasuo Sakurai
Keyword(s):  
Computed Tomography ◽  
Light Chain ◽  
Kidney Biopsy ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition
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