PMID- 11409038
OWN - NLM
STAT- MEDLINE
DCOM- 20011205
LR  - 20191104
IS  - 1350-6129 (Print)
IS  - 1350-6129 (Linking)
VI  - 8
IP  - 2
DP  - 2001 Jun
TI  - Abnormal immunoglobulin synthesis in monoclonal immunoglobulin light chain and 
      light and heavy chain deposition disease.
PG  - 84-93
AB  - The Congo red-binding fibrils of AL amyloidosis are the most common form of 
      monoclonal immunoglobulin tissue deposition (MIDD). Nonetheless, the less 
      structured deposits found in light chain deposition disease (LCDD) and the 
      similar, but distinct, deposits of light and heavy chain deposition disease 
      (LHCDD) and heavy chain deposition disease (HCDD) can produce significant 
      clinical pathology. Analyses of immunoglobulin synthesis by bone marrow cells 
      obtained from 7 patients with LCDD and LHCDD demonstrated the production of 
      excess light chains in all and the presence of incomplete light chains or heavy 
      chain fragments in 5, regardless of the presence of an intact monoclonal protein 
      or related subunit in the serum or urine. Our data indicate that, as is the case 
      with the fibrillar deposits of AL amyloid, the non-fibrillar forms of monoclonal 
      Ig deposition (LCDD and LHCDD) can be associated with the presence of 
      immunoglobulin fragments in bone marrow cells. In some instances these appeared 
      to be synthetic in origin, although rapid intracellular proteolysis or a 
      combination of both could not be excluded. In either case the fragments may be 
      more susceptible to tissue deposition, with subsequent organ compromise, than 
      intact Ig chains.
FAU - Buxbaum, J N
AU  - Buxbaum JN
AD  - Research Service New York V.A. Medical Center, Departments of Medicine and 
      Pathology and Kaplan Cancer Center, New York University School of Medicine, New 
      York City, NY, USA. jbux@scripps.edu
LA  - eng
PT  - Journal Article
PT  - Research Support, U.S. Gov't, Non-P.H.S.
PL  - England
TA  - Amyloid
JT  - Amyloid : the international journal of experimental and clinical investigation : 
      the official journal of the International Society of Amyloidosis
JID - 9433802
RN  - 0 (Antibodies, Monoclonal)
RN  - 0 (Immunoglobulin Fragments)
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulins)
SB  - IM
MH  - Amyloidosis/immunology/*metabolism
MH  - Antibodies, Monoclonal/immunology/metabolism
MH  - Bodily Secretions/immunology/metabolism
MH  - Bone Marrow Cells/immunology/*metabolism
MH  - Cytoplasm/immunology/metabolism
MH  - Heavy Chain Disease/immunology/*metabolism
MH  - Humans
MH  - Immunoglobulin Fragments/immunology/metabolism
MH  - Immunoglobulin Light Chains/immunology/*metabolism
MH  - Immunoglobulins/*biosynthesis/blood/urine
EDAT- 2001/06/21 10:00
MHDA- 2002/01/05 10:01
CRDT- 2001/06/21 10:00
PHST- 2001/06/21 10:00 [pubmed]
PHST- 2002/01/05 10:01 [medline]
PHST- 2001/06/21 10:00 [entrez]
AID - 10.3109/13506120109007349 [doi]
PST - ppublish
SO  - Amyloid. 2001 Jun;8(2):84-93. doi: 10.3109/13506120109007349.