Generalized Primary Amyloid Lymphadenopathy

Jin Hyun Park; Ji Hyun Kwon; Ji Won Kim; Hyeon Jin Cho; Ki Hwan Kim; Doo Hyun Chung; Inho Kim; Sung-Soo Yoon; Seonyang Park; Byoung Kook Kim

doi:10.5045/kjh.2009.44.4.320

Journal List > Korean J Hematol > v.44(4) > 1032856

Go to TopGo to Top Go to BottomGo to Bottom

TOOLS

Park, Kwon, Kim, Cho, Kim, Chung, Kim, Yoon, Park, and Kim: Generalized Primary Amyloid Lymphadenopathy

Case Report

Korean J Hematol 2009;44(4):320-324.

Published online: 20 December 2009

DOI: https://doi.org/10.5045/kjh.2009.44.4.320

Generalized Primary Amyloid Lymphadenopathy

Jin Hyun Park, M.D.², Ji Hyun Kwon, M.D.², Ji Won Kim, M.D.², Hyeon Jin Cho, M.D.², Ki Hwan Kim, M.D.^1,², Doo Hyun Chung, Ph.D.⁴, Inho Kim, Ph.D.^1,^2,³, Sung-Soo Yoon, Ph.D.^1,^2,^3,, Seonyang Park, Ph.D.^1,^2,³, Byoung Kook Kim, Ph.D.^1,^2,³

¹Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea

²Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea

³Clinical Research Institute, Seoul National University Hospital, Seoul, Korea

⁴Department of Pathology, Seoul National University College of Medicine, Seoul, Korea

Correspondence to：Sung-Soo Yoon, M.D., Ph.D. Department of Internal Medicine, Seoul National University College of Medicine 28, Yeongeon-dong, Jongno-gu, Seoul 110-744, Korea Tel: ＋82-2-2072-3079, Fax: ＋82-2-762-9662 E-mail: ssysmc@snu.ac.kr

Received 31 August 2009 Revised 10 December 2009 Accepted 18 December 2009

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Systemic amyloidosis is a disease that displays deposition of insoluble polymeric protein fibrils in tissues and organs. We report here on a case of a 64-year-old woman who initially presented with multiple enlarged lymph nodes. Computed tomography showed multiple enlarged lymph nodes in the mediastinal, lower cervical, supraclavicular, axillary and abdominal areas. Excision biopsy of the cervical lymph nodes and the subsequent histopathology showed amorphous eosinophilic material deposits, and these revealed apple-green birefringence on a polarizing microscopic examination on the Congo-red stained slide. The patient was diagnosed with amyloidosis and she received chemotherapy consisting of melphalan and dexamethasone. During chemotherapy, she was diagnosed with breast cancer. After modified unilateral radical mastectomy, the dexamethasone was restarted and this therapy resulted in stable disease.

Keywords: Systemic amyloidosis, Multiple lymph node enlargement, Chemotherapy

References

1. Westermark P, Benson MD, Buxbaum JN, et al. Amyloid: toward terminology clarification. Report from the nomenclature committee of the international society of amyloidosis. Amyloid. 2005; 12:1–4.

2. Matsuda M, Gono T, Shimojima Y, et al. AL amyloidosis manifesting as systemic lymphadenopathy. Amyloid. 2008; 15:117–24.

3. Wechalekar AD, Hawkins PN, Gillmore JD. Perspectives in treatment of AL amyloidosis. Br J Haematol. 2008; 140:365–77.

4. Leach DB, Hester TO, Farrell HA, Chowdhury K. Primary amyloidosis presenting as massive cervical lymphadenopathy with severe dyspnea: a case report and review of the literature. Otolaryngol Head Neck Surg. 1999; 120:560–4.

5. Ko HS, Davidson JW, Pruzanski W. Amyloid lymphadenopathy. Ann Intern Med. 1976; 85:763–4.

6. Rajkumar SV, Gertz MA. Advances in the treatment of amyloidosis. N Engl J Med. 2007; 356:2413–5.

7. Skinner M, Sanchorawala V, Seldin DC, et al. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med. 2004; 140:85–93.

8. Comenzo RL, Gertz MA. Autologous stem cell transplantation for primary systemic amyloidosis. Blood. 2002; 99:4276–82.

9. Vogel MN, Wehrmann M, Horger MS. Massive cervical and abdominal lymphadenopathy caused by localized amyloidosis. J Clin Oncol. 2007; 25:343–4.

10. Sanchorawala V, Wright DG, Seldin DC, et al. An overview of the use of high-dose melphalan with autologous stem cell transplantation for the treatment of AL amyloidosis. Bone Marrow Transplant. 2001; 28:637–42.

Fig. 1.

Computerized tomography (CT) scans at diagnosis showing multiple lymph node enlargements with high enhancement in the cervical (A), mediastinal (B), and abdominal (C) area.

Fig. 2.

Cervical lymph node biopsy specimens: H & E stain (×10) (A), and H & E stain (×100) (B) showing amorphous eosinophilic material deposits and chronic inflammation with multinucleated giant cells.

Fig. 3.

Congo red stain (×40) of cervical lymph node: Amorphous eosinophilic material deposit (A) and apple-green birefringence (B).

Fig. 4.

Immunohistochemical stain for lambda light chain was positive (A), but negative for kappa light chain (B). (Polymer method, ×400).

TOOLS

Similar articles