Korean J Hematol.  2009 Dec;44(4):320-324. 10.5045/kjh.2009.44.4.320.

Generalized Primary Amyloid Lymphadenopathy

• Park JH
• Kwon JH
• Kim JW
• Cho HJ
• Kim KH
• Chung DH
• Kim I
• Yoon SS
• Park S
• Kim BK

• KMID: 2252120
• DOI: http://doi.org/10.5045/kjh.2009.44.4.320

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.

Keyword

Systemic amyloidosis; Multiple lymph node enlargement; Chemotherapy

MeSH Terms

Amyloid
Amyloidosis
Biopsy
Birefringence
Breast Neoplasms
Dexamethasone
Eosinophils
Female
Humans
Lymph Nodes
Lymphatic Diseases
Mastectomy, Radical
Melphalan
Middle Aged
Polymers
Amyloid
Dexamethasone
Melphalan
Polymers

Figure

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  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.

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  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.

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  Fig. 3. Congo red stain (×40) of cervical lymph node: Amorphous eosinophilic material deposit (A) and apple-green birefringence (B).

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  Fig. 4. Immunohistochemical stain for lambda light chain was positive (A), but negative for kappa light chain (B). (Polymer method, ×400).

Reference

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