PMID- 12358904
OWN - NLM
STAT- MEDLINE
DCOM- 20021213
LR  - 20190705
IS  - 0007-1048 (Print)
IS  - 0007-1048 (Linking)
VI  - 119
IP  - 1
DP  - 2002 Oct
TI  - Tumoral non-amyloidotic monoclonal immunoglobulin light chain deposits 
      ('aggregoma'): presenting feature of B-cell dyscrasia in three cases with 
      immunohistochemical and biochemical analyses.
PG  - 62-9
AB  - Tumoral monoclonal immunoglobulin (Ig) light chain non-fibrillar deposits 
      ('aggregomas'), which can be considered analogous to solitary light chain 
      amyloidomas, are a rare presenting feature of B-cell dyscrasias. It is not 
      certain if they are truly localized or if in reality they represent an initial 
      expression of a silent systemic non-amyloid light chain deposition disease 
      (LCDD). This report describes three patients, two of whom presented with cervical 
      masses and the third with a solitary lung nodule, each comprising granular 
      aggregates of monoclonal kappa light chain. Extracted deposits from the lymph 
      node of one patient were shown by N-terminal amino acid sequence analysis to 
      belong to the variable-region kappa I (Vkappa I) light chain subgroup, the first 
      reported kappa-LCDD protein encoded by the L9 gene and the first report of an 
      expressed protein related to this gene. Extracted deposits from the lung nodule 
      of the second patient belonged to the Vkappa IV light chain subgroup encoded by 
      the B3 germ line gene. The N-terminal amino acid sequences of the light chains 
      from the aggregomas were compared with the related germ line sequences and to the 
      N-terminal amino acid sequences of the nine other known kappa-LCDD light chains 
      reported thus far from patients with systemic LCDD.
FAU - Rostagno, Agueda
AU  - Rostagno A
AD  - Department of Pathology, New York University School of Medicine, and Weill 
      Medical College of Cornell University, New York, NY, USA.
FAU - Frizzera, Glauco
AU  - Frizzera G
FAU - Ylagan, Lourdes
AU  - Ylagan L
FAU - Kumar, Asok
AU  - Kumar A
FAU - Ghiso, Jorge
AU  - Ghiso J
FAU - Gallo, Gloria
AU  - Gallo G
LA  - eng
GR  - AG10491/AG/NIA NIH HHS/United States
GR  - NS38777/NS/NINDS NIH HHS/United States
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - England
TA  - Br J Haematol
JT  - British journal of haematology
JID - 0372544
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulin kappa-Chains)
SB  - IM
MH  - Aged
MH  - Amyloidosis/metabolism
MH  - B-Lymphocytes/*metabolism
MH  - Humans
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Immunoglobulin kappa-Chains/metabolism
MH  - Immunohistochemistry/methods
MH  - Lymph Nodes/metabolism
MH  - Male
MH  - Middle Aged
MH  - Waldenstrom Macroglobulinemia/*metabolism
EDAT- 2002/10/03 04:00
MHDA- 2002/12/17 04:00
CRDT- 2002/10/03 04:00
PHST- 2002/10/03 04:00 [pubmed]
PHST- 2002/12/17 04:00 [medline]
PHST- 2002/10/03 04:00 [entrez]
AID - 3781 [pii]
AID - 10.1046/j.1365-2141.2002.03781.x [doi]
PST - ppublish
SO  - Br J Haematol. 2002 Oct;119(1):62-9. doi: 10.1046/j.1365-2141.2002.03781.x.