PMID- 19203545
OWN - NLM
STAT- MEDLINE
DCOM- 20090331
LR  - 20190608
IS  - 0301-0430 (Print)
IS  - 0301-0430 (Linking)
VI  - 71
IP  - 1
DP  - 2009 Jan
TI  - Clinicopathological features and prognosis in immunoglobulin light and heavy 
      chain deposition disease.
PG  - 9-20
AB  - BACKGROUND: There are three subtypes of monoclonal immunoglobulin deposition 
      disease: light chain deposition disease (LCDD), light and heavy chain deposition 
      disease (LHCDD), and heavy chain deposition disease (HCDD). Although it has been 
      considered that LHCDD is a variant of LCDD, information on clinicopathological 
      features and prognosis in LHCDD is presently limited. METHODS: We reviewed 5,443 
      renal biopsies, and evaluated clinicopathological features and outcomes in 
      patients with LHCDD, in comparison with those in patients with LCDD and 
      previously reported patients with HCDD. We also characterized paraprotein 
      deposits in patients with LHCDD. RESULTS: We identified 6 patients with LHCDD, 6 
      patients with LCDD, and 1 patient with HCDD. The most common clinicopathological 
      findings in patients with LHCDD were proteinuria, renal insufficiency, and 
      nodular sclerosing glomerulopathy. Three patients had IgG-k deposits and 3 
      patients had IgG-l deposits. Heavy chain subclass analysis performed in 4 
      patients showed IgG3 deposits in all patients. Dual immunostaining revealed 
      glomerular colocalization of light and heavy chains. In contrast with LCDD, 
      glomerular C3 and C1q deposits were common findings in LHCDD and HCDD. All 
      patients with LHCDD were treated with steroids and cytotoxic agents, but no 
      effect on proteinuria was observed. Three patients developed end-stage renal 
      disease requiring hemodialysis. The underlying hematological disorders in LHCDD 
      and HCDD were milder than in LCDD. Early renal survival and overall patient 
      survival in our patients appeared to be better in LHCDD than in LCDD. 
      CONCLUSIONS: There are apparent differences in clinicopathological features and 
      prognosis between LHCDD and LCDD. LHCDD is probably more similar to HCDD.
FAU - Masai, R
AU  - Masai R
AD  - Third Department of Internal Medicine, Akita University School of Medicine, Akita 
      Kumiai General Hospital, Akita, Japan.
FAU - Wakui, H
AU  - Wakui H
FAU - Togashi, M
AU  - Togashi M
FAU - Maki, N
AU  - Maki N
FAU - Ohtani, H
AU  - Ohtani H
FAU - Komatsuda, A
AU  - Komatsuda A
FAU - Sawada, K
AU  - Sawada K
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Germany
TA  - Clin Nephrol
JT  - Clinical nephrology
JID - 0364441
RN  - 0 (Immunoglobulin Heavy Chains)
RN  - 0 (Immunoglobulin Light Chains)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Cohort Studies
MH  - Female
MH  - Humans
MH  - *Immunoglobulin Heavy Chains
MH  - *Immunoglobulin Light Chains
MH  - Kidney Diseases/etiology/mortality/pathology
MH  - Male
MH  - Middle Aged
MH  - Paraproteinemias/*diagnosis/mortality/therapy
MH  - Retrospective Studies
MH  - Survival Rate
MH  - Treatment Outcome
EDAT- 2009/02/11 09:00
MHDA- 2009/04/01 09:00
CRDT- 2009/02/11 09:00
PHST- 2009/02/11 09:00 [entrez]
PHST- 2009/02/11 09:00 [pubmed]
PHST- 2009/04/01 09:00 [medline]
AID - 5327 [pii]
AID - 10.5414/cnp71009 [doi]
PST - ppublish
SO  - Clin Nephrol. 2009 Jan;71(1):9-20. doi: 10.5414/cnp71009.