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Monoclonal Immunoglobulin Deposition Disease

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Paraproteinemia and Related Disorders

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Abstract

Monoclonal immunoglobulin deposition disease (MIDD) is a systemic disease characterized by the abnormal clonal production and tissue deposition of monoclonal immunoglobulins (mIg). Three subtypes of MIDD have been reported: light chain deposition disease, which is the most common, light- and heavy-chain deposition disease, and heavy-chain deposition disease. An appropriate diagnostic workup may be challenging, owing to the plethora of available laboratory tests. Renal involvement is the clinical hallmark of MIDD: severe proteinuria (consisting of light chains and other plasma proteins) and renal dysfunction are the most common clinical presentations, albeit in a minority of cases the disease presents with a progressive chronic kidney disease without prominent proteinuria, leading to a diagnostic delay. The affinity of mIg for basement membrane components is thought to be due to the peculiar structural features of the involved pathogenic chain, and the differential involvement of glomeruli, tubules, interstitium, and arteries shapes the clinical picture. Moreover, mIg can deposit in other organs, resulting in a variety of clinical presentations. The natural history and prognosis of MIDD depend on the severity of renal failure and/or heart involvement at diagnosis, the presence of an underlying multiple myeloma, and the delay in the hematologic response to chemotherapy. Current treatment strategies aim to reduce the production of mIg and mainly consist of high-dose melphalan followed by autologous stem cell transplantation or bortezomib-based regimens.

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References

  1. Randall RE, Williamson WC Jr, Mullinax F, Tung MY, Still WJ. Manifestations of systemic light chain deposition. Am J Med. 1976;60(2):293–9.

    CAS  PubMed  Google Scholar 

  2. Cohen C, Royer B, Javaugue V, et al. Bortezomib produces high hematological response rates with prolonged renal survival in monoclonal immunoglobulin deposition disease. Kidney Int. 2015;88(5):1135–43.

    CAS  PubMed  Google Scholar 

  3. Nasr SH, Valeri AM, Cornell LD, et al. Renal monoclonal immunoglobulin deposition disease: a report of 64 patients from a single institution. Clin J Am Soc Nephrol. 2012;7(2):231–9.

    PubMed  Google Scholar 

  4. Sayed RH, Wechalekar AD, Gilbertson JA, et al. Natural history and outcome of light chain deposition disease. Blood. 2015;126(26):2805–10.

    CAS  PubMed  PubMed Central  Google Scholar 

  5. Katzmann JA, Kyle RA, Benson J, et al. Screening panels for detection of monoclonal gammopathies. Clin Chem. 2009;55(8):1517–22.

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Buxbaum JN, Chuba JV, Hellman GC, Solomon A, Gallo GR. Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis. Ann Intern Med. 1990;112:455–64.

    CAS  PubMed  Google Scholar 

  7. Lin J, Markowitz GS, Valeri AM, et al. Renal monoclonal immunoglobulin deposition disease: the disease spectrum. J Am Soc Nephrol. 2001;12(7):1482–92.

    PubMed  Google Scholar 

  8. Pozzi C, D’Amico M, Fogazzi GB, et al. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis. 2003;42(6):1154–63.

    PubMed  Google Scholar 

  9. Joly F, Cohen C, Javaugue V, et al. Randall-type monoclonal immunoglobulin deposition disease: novel insights from a nationwide cohort study. Blood. 2019;133(6):576–87.

    CAS  PubMed  Google Scholar 

  10. Zhang Y, Li X, Liang D, et al. Heavy chain deposition disease: clinicopathologic characteristics of a Chinese case series. Am J Kidney Dis. 2020;75(5):736–43.

    CAS  PubMed  Google Scholar 

  11. Bridoux F, Javaugue V, Bender S, et al. Unravelling the immunopathological mechanisms of heavy chain deposition disease with implications for clinical management. Kidney Int. 2017;91(2):423–34.

    CAS  PubMed  Google Scholar 

  12. Li XM, Rui HC, Liang DD, et al. Clinicopathological characteristics and outcomes of light chain deposition disease: an analysis of 48 patients in a single Chinese center. Ann Hematol. 2016;95(6):901–9.

    PubMed  Google Scholar 

  13. Mohan M, Buros A, Mathur P, et al. Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease. Am J Hematol. 2017;92(8):739–45.

    CAS  PubMed  Google Scholar 

  14. Sicard A, Karras A, Goujon J-M, et al. Light chain deposition disease without glomerular proteinuria: a diagnostic challenge for the nephrologist. Nephrol Dial Transplant. 2014;29(10):1894–902.

    CAS  PubMed  Google Scholar 

  15. Buxbaum J, Gallo G. Nonamyloidotic monoclonal immunoglobulin deposition disease. Light-chain, heavy-chain, and light- and heavy-chain deposition diseases. Hematol Oncol Clin North Am. 1999;13(6):1235–48.

    CAS  PubMed  Google Scholar 

  16. Buxbaum JN, Genega EM, Lazowski P, et al. Infiltrative nonamyloidotic monoclonal immunoglobulin light chain cardiomyopathy: an underappreciated manifestation of plasma cell dyscrasias. Cardiology. 2000;93(4):220–8.

    CAS  PubMed  Google Scholar 

  17. Qian Q, Leung N, Theis JD, Dogan A, Sethi S. Coexistence of myeloma cast nephropathy, light chain deposition disease, and nonamyloid fibrils in a patient with multiple myeloma. Am J Kidney Dis. 2010;56(5):971–6.

    PubMed  Google Scholar 

  18. Zand L, Nasr SH, Gertz MA, et al. Clinical and prognostic differences among patients with light chain deposition disease, myeloma cast nephropathy and both. Leuk Lymphoma. 2015;12:1–8.

    Google Scholar 

  19. Herrera GA. Renal lesions associated with plasma cell dyscrasias: practical approach to diagnosis, new concepts, and challenges. Arch Pathol Lab Med. 2009;133:249.

    PubMed  Google Scholar 

  20. Liapis H, Papadakis I, Nakopoulou L. Nodular glomerulosclerosis secondary to μ heavy chain deposits. Hum Pathol. 2000;31:122.

    CAS  PubMed  Google Scholar 

  21. Markowitz GS. Dysproteinemia and the kidney. Adv Anat Pathol. 2004;11:49.

    PubMed  Google Scholar 

  22. Herrera GA. The contributions of electron microscopy to the understanding and diagnosis of plasma cell dyscrasia-related renal lesions. Med Electron Microsc. 2001;34:1.

    CAS  PubMed  Google Scholar 

  23. Nasr SH, Markowitz GS, Stokes MB, et al. Proliferative glomerulonephritis with monoclonal IgG deposits: a distinct entity mimicking immune-complex glomerulonephritis. Kidney Int. 2004;65:85.

    CAS  PubMed  Google Scholar 

  24. Cohen C, Joly F, Sibille A, et al. Randall-type monoclonal immunoglobulin deposition disease: new insights into the pathogenesis. Diagn Manag Diagn. 2021;11(3):420.

    CAS  Google Scholar 

  25. Kourelis TV, Nasr SH, Dispenzieri A, et al. Outcomes of patients with renal monoclonal immunoglobulin deposition disease. Am J Hematol. 2016;91(11):1123–8.

    CAS  PubMed  Google Scholar 

  26. Leung N, Bridoux F, Batuman V, et al. The evaluation of monoclonal gammopathy of renal significance: a consensus report of the international kidney and monoclonal gammopathy research group. Nat Rev Nephrol. 2019;15(1):45–59.

    PubMed  Google Scholar 

  27. Ivanyi B. Frequency of light chain deposition nephropathy relative to renal amyloidosis and Bence Jones cast nephropathy in a necropsy study of patients with myeloma. Arch Pathol Lab Med. 1990;114(9):986–7.

    CAS  PubMed  Google Scholar 

  28. Ajise OE, Roshal M, Wang L, et al. Clinical utility of morphology, immunohistochemistry, flow cytometry, and FISH analysis in monitoring of plasma cell neoplasms in the bone marrow. J Hematopathol 2016;9:9–18. https://doi.org/10.1007/s12308-015-0264-1.

  29. Samanez C, Domingo A, Cibeira MT, et al. Development of rapidly progressive liver light chain deposition under VAD chemotherapy in multiple myeloma. Eur J Haematol. 2006;76(1):83–5.

    PubMed  Google Scholar 

  30. Brilland B, Sayegh J, Croue A, et al. Recovery from LCDD-associated severe liver cholestasis: a case report and literature review. J Gastrointestin Liver Dis. 2016;25:9–103.

    Google Scholar 

  31. Abdallah AOA, Alapat D, Kaur V, Atrash S. Outcomes of autologous stem cell transplant for cardiac AL-amyloidosis and cardiac light chain deposition disease. J Oncol Pharm Pract. 2020;26(5):1128–33.

    CAS  PubMed  Google Scholar 

  32. Nakamura M, Satoh M, Kowada S, et al. Reversible restrictive cardiomyopathy due to light-chain deposition disease. Mayo Clin Proc. 2002;77:193–6.

    PubMed  Google Scholar 

  33. Mercado JJ, Markert JM, Meador W, et al. Primary CNS nonamyloidogenic light chain deposition disease: case report and brief review. Int J Surg Pathol. 2017;25(8):755–60.

    PubMed  Google Scholar 

  34. Wei P, Tao R, Liu Y, et al. Pulmonary light chain deposition disease: a case series and literature review. Ann Transl Med. 2020;8(9):588.

    CAS  PubMed  PubMed Central  Google Scholar 

  35. Rajapreyar I, Joly J, Tallaj J, et al. Pulmonary vascular disease due to plasma cell Dyscrasia. Mayo Clin Proc. 2021;5(1):210–8.

    Google Scholar 

  36. Ronco PM, Alyanakian MA, Mougenot B, Aucouturier P. Light chain deposition disease: a model of glomerulosclerosis defined at the molecular level. JASN. 2001;12(7):1558–65.

    PubMed  Google Scholar 

  37. De Lazzari M, Fedrigo M, Migliore F, et al. Nonamyloidotic light chain cardiomyopathy: the Arrhythmogenic magnetic resonance pattern. Circulation. 2016;133(14):1421–3.

    PubMed  Google Scholar 

  38. Peng SK, French WJ, Cohen AH, Fausel RE. Light chain cardiomyopathy associated with small-vessel disease. Arch Pathol Lab Med. 1988;112:844–6.

    CAS  PubMed  Google Scholar 

  39. Koopman P, van Dorpe J, Maes B, Dujardin K. Light chain deposition disease as a rare cause of restrictive cardiomyopathy. Acta Cardiol. 2009;64(6):821–4.

    PubMed  Google Scholar 

  40. Toor AA, Ramdane BA, Joseph J, et al. Cardiac nonamyloidotic immunoglobulin deposition disease. Mod Pathol. 2006;19:233–7.

    CAS  PubMed  Google Scholar 

  41. Korbet S, Bonsib S. A case of polyneuropathy and proteinuria. CJASN. 2008;3(2):624–36.

    PubMed  PubMed Central  Google Scholar 

  42. Grassi MP, Clerici F, Perin C, et al. Light chain deposition disease neuropathy resembling amyloid neuropathy in a multiple myeloma patient. Ital J Neurol Sci. 1998;19(4):229–33.

    CAS  PubMed  Google Scholar 

  43. Bhargava P, Rushin JM, Rusnock EJ, et al. Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol. 2007;31(2):267–76.

    PubMed  Google Scholar 

  44. Arrossi AV, Merzianu M, Farver C, et al. Nodular pulmonary light chain deposition disease: an entity associated with Sjögren syndrome or marginal zone lymphoma. J Clin Pathol. 2016;69(6):490–6.

    CAS  PubMed  Google Scholar 

  45. Grainger BT, Issa S, Anderson NE. Primary idiopathic CNS non-amyloidogenic light chain deposition disease complicated by treatment-resistant focal seizure disorder. J Clin Neurosci. 2019;59:313–5.

    PubMed  Google Scholar 

  46. O’Malley JT, D’Agati VD, Sherman WH, Grossman ME. Acquired cutis laxa associated with heavy chain deposition disease involving dermal elastic fibers. JAMA Dermatol. 2014;150(11):1192–6.

    PubMed  Google Scholar 

  47. Palladini G, Dispenzieri A, Gertz MA, et al. New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes. J Clin Oncol. 2012;30:4541–9.

    CAS  PubMed  Google Scholar 

  48. Oe Y, Soma J, Sato H, Ito S. Heavy chain deposition disease: an overview. Clin Exp Nephrol. 2013;17(6):771–8.

    CAS  PubMed  Google Scholar 

  49. Angel-Korman A, Stern L, Angel Y, et al. The role of kidney transplantation in monoclonal Ig deposition disease. Kidney Int Rep. 2020;5(4):485–93.

    PubMed  PubMed Central  Google Scholar 

  50. Heilman RL, Velosa JA, Holley KE, Offord KP, Kyle RA. Long- term follow-up and response to chemotherapy in patients with light-chain deposition disease. Am J Kidney Dis. 1992;20(1):34–41.

    CAS  PubMed  Google Scholar 

  51. Milani P, Basset M, Curci P, et al. Daratumumab in light chain deposition disease: rapid and profound hematologic response preserves kidney function. Blood Adv. 2020;4:1321–4.

    PubMed  PubMed Central  Google Scholar 

  52. Leung N, Lager DJ, Gertz MA, Wilson K, Kanakiriya S, Fervenza FC. Long-term outcome of renal transplantation in light-chain deposition disease. Am J Kidney Dis. 2004;43(1):147–53.

    PubMed  Google Scholar 

  53. Short AK, O'Donoghue DJ, Riad HN, Short CD, Roberts IS. Recurrence of light chain nephropathy in a renal allograft. A case report and review of the literature. Am J Nephrol. 2001;21(3):237–40.

    CAS  PubMed  Google Scholar 

  54. Molina-Andújar A, Tovar N, Cuadrado E, et al. Kidney transplantation in monoclonal immunoglobulin deposition disease: a report of 6 cases. Am J Kidney Dis. 2021;78(5):755–9. S0272-6386(21)00576-X.

    PubMed  Google Scholar 

  55. Royer B, Arnulf B, Martinez F, et al. High dose chemotherapy in light chain or light and heavy chain deposition disease. Kidney Int. 2004;65(2):642–8.

    CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Nephrology, Dialysis and Transplantation Unit, Careggi University Hospital, Florence, Italy

    Marco Allinovi

  2. Nephrology and Dialysis Unit, Santo Stefano Hospital, Prato, Italy

    Fiammetta Ravaglia

  3. Department of Biomedical, Experimental and Clinical Sciences, University of Firenze, Florence, Italy

    Gianmarco Lugli & Augusto Vaglio

  4. Department of Health Science, University of Firenze, Florence, Italy

    Francesco Pegoraro

  5. Nephrology and Dialysis Unit, Meyer Children’s Hospital, Florence, Italy

    Augusto Vaglio

Authors
  1. Marco Allinovi
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  2. Fiammetta Ravaglia
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  3. Gianmarco Lugli
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  4. Francesco Pegoraro
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  5. Augusto Vaglio
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Correspondence to Augusto Vaglio .

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Editors and Affiliations

  1. Rheumatology and Clinical Immunology Unit Internal Medicine Department Faculty of Medicine, Cairo University, Cairo, Egypt

    Gaafar Ragab

  2. Department of Medicine, University of Udine, Udine, Udine, Italy

    Luca Quartuccio

  3. College of Medicine, University of Saskatchewan, Saskatoon, SK, Canada

    Hadi Goubran

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Allinovi, M., Ravaglia, F., Lugli, G., Pegoraro, F., Vaglio, A. (2022). Monoclonal Immunoglobulin Deposition Disease. In: Ragab, G., Quartuccio, L., Goubran, H. (eds) Paraproteinemia and Related Disorders. Springer, Cham. https://doi.org/10.1007/978-3-031-10131-1_10

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  • DOI: https://doi.org/10.1007/978-3-031-10131-1_10

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