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Amyloidosis of the Lymph Nodes and the Spleen

  • Filiz Sen
  • Ahmet DoganEmail author
Part of the Current Clinical Pathology book series (CCPATH)

Abstract

Lymph node and spleen involvement is frequent in systemic amyloidosis. AL amyloidosis is the most common and clinically most important cause of lymph node amyloidosis. In most cases, the lymph node AL amyloidosis is systemic and caused by an underlying systemic plasma cell or B-cell neoplasm. For this reason, immunophenotypic analysis of the lymphoid component is required in these cases for management decisions. Other etiologies of lymph node amyloidosis include AA amyloidosis and rarely hereditary amyloidoses such as AAPOA1 or ATTR amyloidosis. By imaging studies, the spleen is involved in most cases of systemic AL and AA amyloidosis. Clinically splenic involvement may lead to hyposplenism, and occasionally, splenic rupture. In addition to AL and AA, amyloidosis and other systemic amyloidoses such as ALECT2 amyloidosis can also affect the spleen.

Keywords

Amyloidosis Lymph node Spleen Plasma cell neoplasms Myeloma Lymphoplasmacytic lymphoma LECT2 AA SAA 

References

  1. 1.
    Kyle RA, Bayrd ED. Amyloidosis: review of 236 cases. Medicine (Baltimore). 1975;54:271–99.CrossRefGoogle Scholar
  2. 2.
    Atkinson AJ. Primary amyloidosis of liver, spleen, kidneys, suprarenals, bone marrow, pancreas, lymph nodes, and blood vessels in lungs. Gastroenterology. 1946;7:477–82.PubMedGoogle Scholar
  3. 3.
    Bauer W, Jones CM, et al. Primary systemic amyloidosis, involving myocardium, cardiac valves, lungs, spleen, lymph nodes and blood vessels. N Engl J Med. 1949;240:572–7.CrossRefPubMedGoogle Scholar
  4. 4.
    Pilon VA, Gomez LG, Butler JJ. Systemic amyloidosis associated with a benign mesenteric lymphoid mass. Am J Clin Pathol. 1982;78:112–6.PubMedGoogle Scholar
  5. 5.
    Newland JR, Linke RP, Kleinsasser O, Lennert K. Lymph node enlargement due to amyloid. Virchows Arch A Pathol Anat Histopathol. 1983;399:233–6.CrossRefPubMedGoogle Scholar
  6. 6.
    Melato M, Antonutto G, Falconieri G, Manconi R. Massive amyloidosis of mediastinal lymph nodes in a patient with multiple myeloma. Thorax. 1983;38:151–2.PubMedCentralCrossRefPubMedGoogle Scholar
  7. 7.
    Naschitz JE, Yeshurun D, Pick AI. Intrathoracic amyloid lymphadenopathy. Respiration. 1986;49:73–6.CrossRefPubMedGoogle Scholar
  8. 8.
    Newland JR, Linke RP, Lennert K. Amyloid deposits in lymph nodes: a morphologic and immunohistochemical study. Hum Pathol. 1986;17:1245–9.CrossRefPubMedGoogle Scholar
  9. 9.
    Kahn H, Strauchen JA, Gilbert HS, Fuchs A. Immunoglobulin-related amyloidosis presenting as recurrent isolated lymph node involvement. Arch Pathol Lab Med. 1991;115:948–50.PubMedGoogle Scholar
  10. 10.
    Dalton HR, Featherstone T, Athanasou N. Organ limited amyloidosis with lymphadenopathy. Postgrad Med J. 1992;68:47–50.PubMedCentralCrossRefPubMedGoogle Scholar
  11. 11.
    Boss JH. Immunoglobulin-related amyloidosis presenting as recurrent isolated lymph node involvement. Arch Pathol Lab Med. 1993;117:870.PubMedGoogle Scholar
  12. 12.
    Lanzafame S, Magro G, Buffone N, Emmanuele C, Cirino E. Localized primary amyloidosis of axillary lymph nodes. Histopathology. 1996;28:369–71.CrossRefPubMedGoogle Scholar
  13. 13.
    Spitale LS, Jimenez DB, Montenegro RB. Localised primary amyloidosis of inguinal lymph node with superimposed bone metaplasia. Pathology. 1998;30:321–2.CrossRefPubMedGoogle Scholar
  14. 14.
    Magro G, Manusia M, Grasso S. Recurrent isolated amyloid lymphadenopathy due to primary plasmacytoma of lymph nodes. Histopathology. 1999;35:581–2.CrossRefPubMedGoogle Scholar
  15. 15.
    Shi Q, Fan K, Chen H. Localized amyloidosis of cervical lymph nodes. Chin Med J (Engl). 2000;113:184–5.Google Scholar
  16. 16.
    Meeus G, Ponette J, Delabie J, Verschakelen J, Blockmans D. Immunoglobulin related amyloidosis presenting as isolated lymph node and pulmonary involvement. Leuk Lymphoma. 2000;38:423–7.CrossRefPubMedGoogle Scholar
  17. 17.
    Mohanty SK, Arora R, Kakkar N, Varma N, Panda N. Amyloidoma of lymph node. Am J Hematol. 2002;70:177–9.CrossRefPubMedGoogle Scholar
  18. 18.
    Kutlay S, Hasan T, Keven K, Nergizoglu G, Ates K, Karatan O. Primary amyloidosis presenting with massive generalized lymphadenopathy. Leuk Lymphoma. 2002;43:1501–3.CrossRefPubMedGoogle Scholar
  19. 19.
    Bielsa S, Jover A, Porcel JM. Isolated lymph node amyloidosis. Eur J Intern Med. 2005;16:619.CrossRefPubMedGoogle Scholar
  20. 20.
    Biewend ML, Menke DM, Calamia KT. The spectrum of localized amyloidosis: a case series of 20 patients and review of the literature. Amyloid. 2006;13:135–42.CrossRefPubMedGoogle Scholar
  21. 21.
    Yong HS, Woo OH, Lee JW, Suh SI, Oh YW, Kang EY. Primary localized amyloidosis manifested as supraclavicular and mediastinal lymphadenopathy. Br J Radiol. 2007;80:e131–3.CrossRefPubMedGoogle Scholar
  22. 22.
    Leiro V, Fernandez-Villar A, Bandres R, Gonzalez A, Represas C, Barros JC, et al. Primary amyloidosis involving mediastinal lymph nodes: diagnosis by transbronchial needle aspiration. Respiration. 2008;76:218–20.CrossRefPubMedGoogle Scholar
  23. 23.
    Terrier B, Jaccard A, Harousseau JL, Delarue R, Tournilhac O, Hunault-Berger M, et al. The clinical spectrum of IgM-related amyloidosis: a French nationwide retrospective study of 72 patients. Medicine (Baltimore). 2008;87:99–109.CrossRefGoogle Scholar
  24. 24.
    Wechalekar AD, Lachmann HJ, Goodman HJ, Bradwell A, Hawkins PN, Gillmore JD. AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome. Blood. 2008;112:4009–16.CrossRefPubMedGoogle Scholar
  25. 25.
    Matsuda M, Gono T, Shimojima Y, Yoshida T, Katoh N, Hoshii Y, et al. AL amyloidosis manifesting as systemic lymphadenopathy. Amyloid. 2008;15:117–24.CrossRefPubMedGoogle Scholar
  26. 26.
    Sharma S, Kotru M, Gupta R. Isolated amyloidosis of cervical lymph nodes. Acta Cytol. 2010;54:1078–80.CrossRefPubMedGoogle Scholar
  27. 27.
    Seccia V, Dallan I, Cervetti G, Lenzi R, Marchetti M, Casani AP, et al. A rare case of primary systemic amyloidosis of the neck with massive cervical lymph node involvement: a case report and review of the literature. Leuk Res. 2010;34:e100–3.CrossRefPubMedGoogle Scholar
  28. 28.
    Matsuda M, Katoh N, Tazawa K, Shimojima Y, Mishima Y, Sano K, et al. Surgical removal of amyloid-laden lymph nodes: a possible therapeutic approach in a primary systemic AL amyloidosis patient with focal lymphadenopathy. Amyloid. 2011;18:79–82.CrossRefPubMedGoogle Scholar
  29. 29.
    Chan T, Mak HK, Kwong YL. Generalized lymph node immunoglobulin G amyloidoma. Ann Hematol. 2012;91:1503–4.PubMedCentralCrossRefPubMedGoogle Scholar
  30. 30.
    D’Souza A, Theis J, Quint P, Kyle R, Gertz M, Zeldenrust S, et al. Exploring the amyloid proteome in immunoglobulin-derived lymph node amyloidosis using laser microdissection/tandem mass spectrometry. Am J Hematol. 2013;88:577–80.CrossRefPubMedGoogle Scholar
  31. 31.
    Fu J, Seldin DC, Berk JL, Sun F, O’Hara C, Cui H, et al. Lymphadenopathy as a manifestation of amyloidosis: a case series. Amyloid. 2014;21:256–60.CrossRefPubMedGoogle Scholar
  32. 32.
    Bhavsar T, Vincent G, Durra H, Khurana JS, Huang Y. Primary amyloidosis involving mesenteric lymph nodes: diagnosis by fine-needle aspiration cytology. Acta Cytol. 2011;55:296–301.CrossRefPubMedGoogle Scholar
  33. 33.
    Telio D, Bailey D, Chen C, Crump M, Reece D, Kukreti V. Two distinct syndromes of lymphoma associated AL amyloidosis: a case series and review of the literature. Am J Hematol. 2010;85:805–8.CrossRefPubMedGoogle Scholar
  34. 34.
    Simmonds PD, Cottrell BJ, Mead GM, Wright DH, Whitehouse JMA. Lymphadenopathy due to amyloid deposition in non-Hodgkin’s lymphoma. Ann Oncol. 1997;8:267–70.CrossRefPubMedGoogle Scholar
  35. 35.
    Cohen AD, Zhou P, Xiao Q, Fleisher M, Kalakonda N, Akhurst T, et al. Systemic AL amyloidosis due to non-Hodgkin’s lymphoma: an unusual clinicopathologic association. Br J Haematol. 2004;124:309–14.CrossRefPubMedGoogle Scholar
  36. 36.
    Palladini G, Russo P, Bosoni T, Sarais G, Lavatelli F, Foli A, et al. AL amyloidosis associated with IgM monoclonal protein: a distinct clinical entity. Clin Lymphoma Myeloma. 2009;9:80–3.CrossRefPubMedGoogle Scholar
  37. 37.
    Eriksson M, Schonland S, Yumlu S, Hegenbart U, von Hutten H, Gioeva Z, et al. Hereditary apolipoprotein AI-associated amyloidosis in surgical pathology specimens: identification of three novel mutations in the APOA1 gene. J Mol Diagn. 2009;11:257–62.PubMedCentralCrossRefPubMedGoogle Scholar
  38. 38.
    Rostagno A, Frizzera G, Ylagan L, Kumar A, Ghiso J, Gallo G. Tumoral non-amyloidotic monoclonal immunoglobulin light chain deposits (‘aggregoma’): presenting feature of B-cell dyscrasia in three cases with immunohistochemical and biochemical analyses. Br J Haematol. 2002;119:62–9.CrossRefPubMedGoogle Scholar
  39. 39.
    Schonland SO, Hegenbart U, Bochtler T, Mangatter A, Hansberg M, Ho AD, et al. Immunohistochemistry in the classification of systemic forms of amyloidosis: a systematic investigation of 117 patients. Blood. 2012;119:488–93.CrossRefPubMedGoogle Scholar
  40. 40.
    Vrana JA, Gamez JD, Madden BJ, Theis JD, Bergen III HR, Dogan A. Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens. Blood. 2009;114:4957–9.CrossRefPubMedGoogle Scholar
  41. 41.
    Hawkins PN, Lavender JP, Pepys MB. Evaluation of systemic amyloidosis by scintigraphy with 123I-labeled serum amyloid P component. N Engl J Med. 1990;323:508–13.CrossRefPubMedGoogle Scholar
  42. 42.
    Stone MJ. Functional hyposplenism and amyloidosis. Am J Clin Pathol. 1982;78:570.PubMedGoogle Scholar
  43. 43.
    Boyko WJ, Pratt R, Wass H. Functional hyposplenism, a diagnostic clue in amyloidosis. Report of six cases. Am J Clin Pathol. 1982;77:745–8.PubMedGoogle Scholar
  44. 44.
    Gertz MA, Kyle RA, Greipp PR. Hyposplenism in primary systemic amyloidosis. Ann Intern Med. 1983;98:475–7.CrossRefPubMedGoogle Scholar
  45. 45.
    Selby CD, Sprott VM, Toghill PJ. Impaired splenic function in systemic amyloidosis. Postgrad Med J. 1987;63:357–60.PubMedCentralCrossRefPubMedGoogle Scholar
  46. 46.
    Powsner RA, Simms RW, Chudnovsky A, Lee VW, Skinner M. Scintigraphic functional hyposplenism in amyloidosis. J Nucl Med. 1998;39:221–3.PubMedGoogle Scholar
  47. 47.
    King FH, Oppenheimer GD. Rupture of amyloid spleen. Ann Intern Med. 1948;29:374–8.CrossRefPubMedGoogle Scholar
  48. 48.
    Wiley AT, Teeter RR, Schnabel Sr TG. Rupture of the spleen in primary amyloidosis; report of a case. Med Clin North Am. 1951;35:1841–7.PubMedGoogle Scholar
  49. 49.
    Drapiewski JF, Sternlieb SB, Jones R. Primary amyloidosis with spontaneous rupture of the spleen and sudden death. Ann Intern Med. 1955;43:406–12.CrossRefPubMedGoogle Scholar
  50. 50.
    Wilson H, Yawn DH. Rupture of the spleen in amyloidosis. JAMA. 1979;241:790–1.CrossRefPubMedGoogle Scholar
  51. 51.
    Cubo T, Ramia JM, Pardo R, Martin J, Padilla D, Hernandez-Calvo J. Spontaneous rupture of the spleen in amyloidosis. Am J Emerg Med. 1997;15:443–4.CrossRefPubMedGoogle Scholar
  52. 52.
    Gupta R, Singh G, Bose SM, Vaiphei K, Radotra B. Spontaneous rupture of the amyloid spleen: a report of two cases. J Clin Gastroenterol. 1998;26:161.CrossRefPubMedGoogle Scholar
  53. 53.
    Oran B, Wright DG, Seldin DC, McAneny D, Skinner M, Sanchorawala V. Spontaneous rupture of the spleen in AL amyloidosis. Am J Hematol. 2003;74:131–5.CrossRefPubMedGoogle Scholar
  54. 54.
    Aydinli B, Ozturk G, Balik AA, Aslan S, Erdogan F. Spontaneous rupture of the spleen in secondary amyloidosis: a patient with rheumatoid arthritis. Amyloid. 2006;13:160–3.CrossRefPubMedGoogle Scholar
  55. 55.
    Chau EM, Chan AC, Chan WK. Spontaneous atraumatic rupture of a normal-sized spleen due to AL amyloid angiopathy. Amyloid. 2008;15:213–5.CrossRefPubMedGoogle Scholar
  56. 56.
    Skok P, Knehtl M, Ceranic D, Glumbic I. Splenic rupture in systemic amyloidosis—case presentation and review of the literature. Z Gastroenterol. 2009;47:292–5.CrossRefPubMedGoogle Scholar
  57. 57.
    Baez-Garcia Jde J, Martinez-Hernandez Magro P, Iriarte-Gallego G, Baez-Avina JA. Spontaneous rupture of the spleen secondary to amyloidosis. Cir Cir. 2010;78:533–7.PubMedGoogle Scholar
  58. 58.
    Shobeiri H, Einakchi M, Khajeh M, Motie MR. Spontaneous rupture of the spleen secondary to amyloidosis. J Coll Physicians Surg Pak. 2013;23:427–9.PubMedGoogle Scholar
  59. 59.
    Solomon A, Macy SD, Wooliver C, Weiss DT, Westermark P. Splenic plasma cells can serve as a source of amyloidogenic light chains. Blood. 2009;113:1501–3.PubMedCentralCrossRefPubMedGoogle Scholar
  60. 60.
    Dogan A, Theis JD, Vrana JA, Jimenez-Zepeda VH, Lacy MQ, Leung N, et al. Clinical and pathological phenotype of leukocyte cell-derived chemotaxin-2 (LECT2) amyloidosis (ALECT2). Amyloid. 2010;17:69–70.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  1. 1.Department of PathologyMemorial Sloan Kettering Cancer CenterNew YorkUSA
  2. 2.Departments of Pathology and Laboratory MedicineMemorial Sloan Kettering Cancer CenterNew YorkUSA

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