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Light and heavy chain deposition disease

MedGen UID:
1684811
Concept ID:
C5204117
Neoplastic Process
Synonyms: LHCDD; Light and Heavy Chain Deposition Disease; light and heavy chain deposition disease
 
Monarch Initiative: MONDO:0019729
Orphanet: ORPHA93557

Definition

A rare non-amyloid monoclonal immunoglobulin deposition disease characterized by secretion of abnormal light and heavy chains, which are deposited in tissues and cause organ dysfunction, but do not form amyloid beta-pleated sheets or contain an amyloid P component. The condition most frequently occurs in association with multiple myeloma. The kidneys are most commonly affected (clinically manifesting as nephrotic syndrome and renal failure), but liver, heart, peripheral nerves, blood vessels, and joints may also be involved. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLight and heavy chain deposition disease

Professional guidelines

PubMed

Suzuki K
Intern Med 2005 Sep;44(9):915-6. doi: 10.2169/internalmedicine.44.915. PMID: 16258202
Sanders PW
Curr Opin Nephrol Hypertens 2005 Mar;14(2):97-103. doi: 10.1097/00041552-200503000-00003. PMID: 15687834

Recent clinical studies

Etiology

Nasr SH, Valeri AM, Cornell LD, Fidler ME, Sethi S, D'Agati VD, Leung N
Clin J Am Soc Nephrol 2012 Feb;7(2):231-9. Epub 2011 Dec 8 doi: 10.2215/CJN.08640811. PMID: 22156754
Masai R, Wakui H, Togashi M, Maki N, Ohtani H, Komatsuda A, Sawada K
Clin Nephrol 2009 Jan;71(1):9-20. doi: 10.5414/cnp71009. PMID: 19203545
Sanders PW
Curr Opin Nephrol Hypertens 2005 Mar;14(2):97-103. doi: 10.1097/00041552-200503000-00003. PMID: 15687834
Royer B, Arnulf B, Martinez F, Roy L, Flageul B, Etienne I, Ronco P, Brouet JC, Fermand JP
Kidney Int 2004 Feb;65(2):642-8. doi: 10.1111/j.1523-1755.2004.00427.x. PMID: 14717936
Buxbaum JN, Chuba JV, Hellman GC, Solomon A, Gallo GR
Ann Intern Med 1990 Mar 15;112(6):455-64. doi: 10.7326/0003-4819-76-3-112-6-455. PMID: 2106817

Diagnosis

Shimamoto Y, Takahashi N, Katoh N, Matsui Y, Mochizuki Y, Ito M, Yazaki M, Kametani F, Kasuno K, Sekijima Y, Naiki H, Iwano M
BMC Nephrol 2023 Jun 26;24(1):187. doi: 10.1186/s12882-023-03207-0. PMID: 37365566Free PMC Article
Masai R, Wakui H, Togashi M, Maki N, Ohtani H, Komatsuda A, Sawada K
Clin Nephrol 2009 Jan;71(1):9-20. doi: 10.5414/cnp71009. PMID: 19203545
Sanders PW
Curr Opin Nephrol Hypertens 2005 Mar;14(2):97-103. doi: 10.1097/00041552-200503000-00003. PMID: 15687834
Daliani D, Weber D, Alexanian R
Am J Hematol 1995 Dec;50(4):296-8. doi: 10.1002/ajh.2830500413. PMID: 7485106
Buxbaum J
Hematol Oncol Clin North Am 1992 Apr;6(2):323-46. PMID: 1582976

Therapy

Hassoun H, Flombaum C, D'Agati VD, Rafferty BT, Cohen A, Klimek VM, Boruchov A, Kewalramani T, Reich L, Nimer SD, Comenzo RL
Bone Marrow Transplant 2008 Sep;42(6):405-12. Epub 2008 Jun 23 doi: 10.1038/bmt.2008.179. PMID: 18574442
Satirapoj B, Paueksakon P
J Med Assoc Thai 2007 Oct;90(10):2204-8. PMID: 18041443
Nakatsuka A, Maeshima Y, Sarai A, Yanai H, Sugiyama H, Yamasaki Y, Makino H
Clin Nephrol 2005 Sep;64(3):221-7. doi: 10.5414/cnp64221. PMID: 16175947
Sanders PW
Curr Opin Nephrol Hypertens 2005 Mar;14(2):97-103. doi: 10.1097/00041552-200503000-00003. PMID: 15687834
Royer B, Arnulf B, Martinez F, Roy L, Flageul B, Etienne I, Ronco P, Brouet JC, Fermand JP
Kidney Int 2004 Feb;65(2):642-8. doi: 10.1111/j.1523-1755.2004.00427.x. PMID: 14717936

Prognosis

Nasr SH, Valeri AM, Cornell LD, Fidler ME, Sethi S, D'Agati VD, Leung N
Clin J Am Soc Nephrol 2012 Feb;7(2):231-9. Epub 2011 Dec 8 doi: 10.2215/CJN.08640811. PMID: 22156754
Masai R, Wakui H, Togashi M, Maki N, Ohtani H, Komatsuda A, Sawada K
Clin Nephrol 2009 Jan;71(1):9-20. doi: 10.5414/cnp71009. PMID: 19203545
Satirapoj B, Paueksakon P
J Med Assoc Thai 2007 Oct;90(10):2204-8. PMID: 18041443
Nakatsuka A, Maeshima Y, Sarai A, Yanai H, Sugiyama H, Yamasaki Y, Makino H
Clin Nephrol 2005 Sep;64(3):221-7. doi: 10.5414/cnp64221. PMID: 16175947
Buxbaum JN, Chuba JV, Hellman GC, Solomon A, Gallo GR
Ann Intern Med 1990 Mar 15;112(6):455-64. doi: 10.7326/0003-4819-76-3-112-6-455. PMID: 2106817

Clinical prediction guides

Masai R, Wakui H, Togashi M, Maki N, Ohtani H, Komatsuda A, Sawada K
Clin Nephrol 2009 Jan;71(1):9-20. doi: 10.5414/cnp71009. PMID: 19203545
Nakatsuka A, Maeshima Y, Sarai A, Yanai H, Sugiyama H, Yamasaki Y, Makino H
Clin Nephrol 2005 Sep;64(3):221-7. doi: 10.5414/cnp64221. PMID: 16175947
Royer B, Arnulf B, Martinez F, Roy L, Flageul B, Etienne I, Ronco P, Brouet JC, Fermand JP
Kidney Int 2004 Feb;65(2):642-8. doi: 10.1111/j.1523-1755.2004.00427.x. PMID: 14717936
Nasr SH, Markowitz GS, Stokes MB, Seshan SV, Valderrama E, Appel GB, Aucouturier P, D'Agati VD
Kidney Int 2004 Jan;65(1):85-96. doi: 10.1111/j.1523-1755.2004.00365.x. PMID: 14675039

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