Light chain storage disease

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Classification according to ICD-10
D89.8 Other specified disorders involving the immune system, not elsewhere classified
ICD-10 online (WHO version 2019)

The light chain storage disease is a rare storage disease with deposits of monoclonal light chains (immunoglobulins) in various organs, especially the kidneys, and is classified by the WHO as one of the “monoclonal deposition diseases”. In contrast to AL amyloidosis, it is not deposited in amyloid fibrils . Synonyms are: light chain deposit disease; Light chain disease; English light-chain deposition disease; LCDD The first description comes from 1976 by the US doctors Russell E. Randall et al .

distribution

The frequency is not known, the median age at diagnosis is 58 years, and males are more frequently affected. Light chain storage disease is found at autopsy in approximately 5% of patients with multiple myeloma . There is an association with multiple myeloma in 50-60% and in 17% with monoclonal gammopathy of unclear significance .

Clinical manifestations

Clinical criteria are:

Differential diagnosis

A distinction must be made between monoclonal gammopathy of renal significance and AL amyloidosis . With regard to the examination methods, the diagnosis basically comprises the methods customary for monoclonal gammopathies (see sections Diagnostic examinations in the article monoclonal gammopathy of renal significance , examination methods in the article AL amyloidosis , diagnostic examinations in the article monoclonal gammopathy of unclear significance and diagnosis in the article multiple myeloma ).

therapy

Treatment options include autologous stem cell transplantation , treatment with bortezomib , modulating immunotherapy, or a kidney transplant .

literature

  • M. Malphettes, P. Bonnet, P. Schneider, M. Vignon, M. Baron, JD Bouaziz, M. Battistella: Light Chain Deposition Disease with Bullous Skin Lesions Mimicking Atypical Bullous Pemphigoid. In: Acta dermato-venereologica. Volume 98, number 1, 01 2018, pp. 140-141, doi: 10.2340 / 00015555-2775 , PMID 29135016 .
  • SV Rajkumar: Pathogenesis of immunoglobulin light chain (AL) amyloidosis and light and heavy chain deposition diseases. In: UpToDate, July 2016, article
  • SV Rajkumar: Clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain (AL) amyloidosis (primary amyloidosis). In: UpToDate, July 2016, article

Individual evidence

  1. ↑ SH Swerdlow, E. Campo et al. a .: The 2016 revision of the World Health Organization classification of lymphoid neoplasms. In: Blood. 127, 2016, p. 2375, doi: 10.1182 / blood-2016-01-643569 .
  2. ↑ Light chain storage disease. In: Orphanet (Rare Disease Database).
  3. ↑ MJ Kim, JH Kim, IY Kim, SB Lee, IS Park, MY Han, H. Rhee, SH Song, EY Seong, IS Kwak, DW Lee: Light Chain Deposition Disease Associated With Multiple Myeloma Developing in Late Pregnancy. In: Iranian journal of kidney diseases. Volume 12, Number 2, 03 2018, pp. 132-134, PMID 29507277 .
  4. ↑ PM Ronco, MA Alyanakian, B. Mougenot, P. Aucouturier: Light chain deposition disease: a model of glomerulosclerosis defined at the molecular level. In: Journal of the American Society of Nephrology: JASN. Volume 12, Number 7, July 2001, pp. 1558-1565, PMID 11423587 (review).
  5. ^ RE Randall, WC Williamson, F. Mullinax, MY Tung, WJ Still: Manifestations of systemic light chain deposition. In: The American journal of medicine. Volume 60, Number 2, February 1976, pp. 293-299, PMID 814812 (Review).
  6. ^ C. Pozzi, M. D'Amico, GB Fogazzi, S. Curioni, F. Ferrario, S. Pasquali, G. Quattrocchio, C. Rollino, S. Segagni, F. Locatelli: Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. In: American Journal of Kidney Diseases . Volume 42, Number 6, December 2003, pp. 1154-1163, PMID 14655186 (review).
  7. ↑ a b c d S. Boppana, RA Sacher: Light-Chain Deposition Disease. Medscape Reference. June 17, 2014; emedicine
  8. ^ Rare Diseases