Ann Rehabil Med.  2017 Oct;41(5):887-891. 10.5535/arm.2017.41.5.887.

Lymphedema Associated With Primary Amyloidosis: A Case Study

• Park G
• Jeong HW
• Lee J
• Mun YC
• Sung SH
• Han SJ

• KMID: 2440818
• DOI: http://doi.org/10.5535/arm.2017.41.5.887

Abstract

We reported on a 60-year-old man presenting lymphedema of both lower extremities and scrotum for 3 years with unknown cause. We took a computed tomography scan of the lower extremities as a follow-up. There were diffuse subcutaneous edema in both lower extremities and multiple enlarged lymph nodes along the para-aortic and bilateral inguinal areas. For further evaluation, biopsy of an enlarged inguinal lymph node was taken, yielding a diagnosis of primary amyloidosis. A treatment of chemotherapy for amyloidosis was recommended for him. To our knowledge, this is the first report of lymphedema presenting with primary amyloidosis in Asia. This case suggests that primary amyloidosis could be one of the differential diagnoses in patients with lymphedema in the lower extremities.

Keyword

Lymphedema; Amyloidosis; Lymph nodes

MeSH Terms

Amyloidosis*
Asia
Biopsy
Diagnosis
Diagnosis, Differential
Drug Therapy
Edema
Follow-Up Studies
Humans
Lower Extremity
Lymph Nodes
Lymphedema*
Middle Aged
Scrotum

Figure

• 

  Fig. 1 Compared with previous lower extremities computed tomography (CT) scan taken 3 years ago (A, arrows), lymph nodes along the para-aortic and bilateral inguinal areas were enlarged at admission (B, arrowheads). The lower extremities CT showed subcutaneous edema in both lower extremities (C, E), and it was aggravated in follow-up CT (D, F). (C, D) above knee 10 cm, (E, F) below knee 10 cm.

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  Fig. 2 Amyloid deposits were found in hematoxylin & eosin (H&E) and Congo red staining of specimens from lymph nodes at diagnosis (histological section). Perivascular amorphous hyaline deposition and thickening of vessel walls on H&E staining (A) and apple-green birefringence on Congo red histological staining (B) were observed.

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  Fig. 3 Severe swelling, redness, and discharges with foul odor were exhibited at admission (A). After 4 weeks of treatment, symptoms had improved (B).

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  Fig. 4 In lymphoscintigraphy obtained 1 hour after injection of radionuclide there was no visualization of main lymphatics in left lower extremity. Decreased lymphatic drainage and clearance of tracer was seen in right lower extremity. Rt, right; Lt, left; ANT, anterior view; POS, posterior view; 1HR, 1 hour after injection of radionuclide.

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