PMID- 37887262
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20231029
IS  - 2305-6320 (Print)
IS  - 2305-6320 (Electronic)
IS  - 2305-6320 (Linking)
VI  - 10
IP  - 10
DP  - 2023 Oct 4
TI  - Monoclonal Gammopathy of Renal Significance with Deposits of Infrequent 
      Morphology: Two Case Reports of Light and Heavy Chain Deposition Disease with 
      Atypical Presentation and Literature Review.
LID - 10.3390/medicines10100055 [doi]
LID - 55
AB  - BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) includes three 
      entities: light chain deposition disease (LCDD), heavy chain deposition disease 
      (HCDD) and light and heavy chain deposition disease (LHCDD). The renal 
      presentation can manifest with varying degrees of proteinuria and/or nephrotic 
      syndrome, microhematuria, and often leads to end-stage renal disease. Given the 
      rarity of LHCDD, therapeutic approaches for this condition remain inconclusive, 
      as clinical trials are limited. CASE PRESENTATION: We report two male patients 
      with underlying monoclonal gammopathy of renal significance (MGRS) associated 
      with LHCDD lesions. Both cases had non-nephrotic proteinuria, moderately impaired 
      renal function, and normal levels of C3 and C4. Light microscopy of the renal 
      biopsies in both patients did not show lesions of nodular glomerulosclerosis. 
      Immunofluorescence showed a staining pattern with interrupted linear IgA-kappa in 
      patient #1 and IgA-lambda in patient #2 only along the glomerular basement membrane 
      (GBM). Electron microscopy of patient #1 revealed electrodense deposits in the 
      subendothelial and mesangial areas only along the GBM. DISCUSSION: In this case 
      series, we discuss the clinical, analytical, and histopathological findings of 
      two rare cases of LHCDD. Both patients exhibited IgA monoclonality and were 
      diagnosed with monoclonal gammopathy of undetermined significance (MGUS) by the 
      hematology department at the time of renal biopsy. Treatment with steroids and 
      cytotoxic agents targeting the clone cells responsible for the deposition disease 
      resulted in a favorable renal and hematologic response.
FAU - De La Flor, Jose C
AU  - De La Flor JC
AUID- ORCID: 0000-0001-9346-2279
AD  - Department of Nephrology, Hospital Central de la Defensa, 28046 Madrid, Spain.
FAU - Monroy-Condori, Maribel
AU  - Monroy-Condori M
AD  - Section of Nephrology and Hypertension, Hospital General Universitario Nuestra 
      Senora del Prado, 45600 Talavera de la Reina, Spain.
FAU - Apaza-Chavez, Jacqueline
AU  - Apaza-Chavez J
AD  - Department of Nephrology, Hospital Fuensanta, 28027 Madrid, Spain.
FAU - Arenas-Moncaleano, Ivan
AU  - Arenas-Moncaleano I
AD  - Section of Nephrology and Hypertension, Hospital General Universitario Nuestra 
      Senora del Prado, 45600 Talavera de la Reina, Spain.
FAU - Diaz, Francisco
AU  - Diaz F
AUID- ORCID: 0000-0001-5090-8488
AD  - Department of Anatomic Pathology, Hospital Gregorio Maranon, 28007 Madrid, Spain.
FAU - Guerra-Torres, Xavier E
AU  - Guerra-Torres XE
AD  - Section of Nephrology and Hypertension, Hospital General Universitario Nuestra 
      Senora del Prado, 45600 Talavera de la Reina, Spain.
FAU - Morales-Montoya, Jorge L
AU  - Morales-Montoya JL
AD  - Section of Nephrology and Hypertension, Hospital General Universitario Nuestra 
      Senora del Prado, 45600 Talavera de la Reina, Spain.
FAU - Lerma-Verdejo, Ana
AU  - Lerma-Verdejo A
AD  - Department of Hematology, Hospital General Nuestra Senora del Prado, 45600 
      Talavera de la Reina, Spain.
FAU - Sandoval, Edna
AU  - Sandoval E
AD  - Department of Hematology, Hospital Central de la Defensa, 28046 Madrid, Spain.
FAU - Villa, Daniel
AU  - Villa D
AUID- ORCID: 0000-0001-8982-8442
AD  - Department of Nephrology, Hospital Clinica Universiad Navarra, 31009 Pamplona, 
      Spain.
FAU - Vieru, Coca-Mihaela
AU  - Vieru CM
AD  - Department of Anatomic Pathology, Hospital Gregorio Maranon, 28007 Madrid, Spain.
LA  - eng
PT  - Case Reports
DEP - 20231004
PL  - Switzerland
TA  - Medicines (Basel)
JT  - Medicines (Basel, Switzerland)
JID - 101671069
PMC - PMC10608252
OTO - NOTNLM
OT  - light and heavy chain deposition disease
OT  - monoclonal gammopathy of renal significance
OT  - monoclonal immunoglobulin deposits disease
COIS- The authors declare no conflict of interest.
EDAT- 2023/10/27 12:43
MHDA- 2023/10/27 12:44
PMCR- 2023/10/04
CRDT- 2023/10/27 06:56
PHST- 2023/07/21 00:00 [received]
PHST- 2023/09/02 00:00 [revised]
PHST- 2023/09/26 00:00 [accepted]
PHST- 2023/10/27 12:44 [medline]
PHST- 2023/10/27 12:43 [pubmed]
PHST- 2023/10/27 06:56 [entrez]
PHST- 2023/10/04 00:00 [pmc-release]
AID - medicines10100055 [pii]
AID - medicines-10-00055 [pii]
AID - 10.3390/medicines10100055 [doi]
PST - epublish
SO  - Medicines (Basel). 2023 Oct 4;10(10):55. doi: 10.3390/medicines10100055.