PMID- 26019820
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20150528
LR  - 20201001
IS  - 2048-8505 (Print)
IS  - 2048-8513 (Electronic)
IS  - 2048-8505 (Linking)
VI  - 5
IP  - 5
DP  - 2012 Oct
TI  - Indolent systemic mastocytosis associated with light chain deposition disease.
PG  - 424-7
LID - 10.1093/ckj/sfs104 [doi]
AB  - Systemic mastocytosis (SM) is characterized by infiltration of neoplastic mast 
      cells in one or more organ systems. SM in association with plasma cell dyscrasia 
      is very rare. We report a first case of indolent SM (ISM) associated with light 
      chain deposition disease (LCDD) in a kidney biopsy from a 59-year-old female 
      presenting with skin rash, elevated serum creatinine, hematuria and mild 
      proteinuria. Subsequent workup demonstrated IgG kappa monoclonal protein in serum 
      and urine. A bone marrow biopsy revealed neoplastic mast cells involving bone 
      marrow without evidence of clonal myeloid or lymphoid proliferation. Kidney 
      biopsy demonstrated modest mesangial expansion detected by light microscopy and 
      unequivocal evidence of monoclonal kappa light chain deposition within glomerular 
      capillaries, tubular basement membranes and vascular walls detected by 
      immunofluorescence and/or electron microscopy, along with equivocal evidence of 
      light chain cast nephropathy. Despite treatment with bortezomib and 
      dexamethasone, her renal function was progressively declined over the next 6 
      months. This case is a reminder that SM can coincide with LCDD, which requires 
      clinical suspicion and multimodality workup on a kidney biopsy including 
      immunofluorescence and electron microscopy to reach the correct diagnosis.
FAU - Sasaki, Kotaro
AU  - Sasaki K
AD  - Department of Pathology , University of Washington Medical Center , Seattle, WA 
      98195 , USA.
FAU - Chang, Alice
AU  - Chang A
AD  - Nephrology , Group Health Cooperative , Seattle, WA 98112 , USA.
FAU - Najafian, Behzad
AU  - Najafian B
AD  - Department of Pathology , University of Washington Medical Center , Seattle, WA 
      98195 , USA.
LA  - eng
PT  - Journal Article
PL  - England
TA  - Clin Kidney J
JT  - Clinical kidney journal
JID - 101579321
PMC - PMC4432416
OTO - NOTNLM
OT  - light chain deposition disease
OT  - systemic mastocytosis
EDAT- 2012/10/01 00:00
MHDA- 2012/10/01 00:01
PMCR- 2012/10/01
CRDT- 2015/05/29 06:00
PHST- 2012/06/15 00:00 [received]
PHST- 2012/07/17 00:00 [accepted]
PHST- 2015/05/29 06:00 [entrez]
PHST- 2012/10/01 00:00 [pubmed]
PHST- 2012/10/01 00:01 [medline]
PHST- 2012/10/01 00:00 [pmc-release]
AID - sfs104 [pii]
AID - 10.1093/ckj/sfs104 [doi]
PST - ppublish
SO  - Clin Kidney J. 2012 Oct;5(5):424-7. doi: 10.1093/ckj/sfs104.