Unilateral conjunctival AL kappa amyloidosis with trace evidence of systemic amyloidosis

Abstract

Background: Amyloidosis is a systemic disorder that results from the tissue deposition of various proteins with distinctive morphological characteristics. Conjunctival amyloidosis is a rare variant which is generally localized and not associated with systemic involvement.

Case report: We present here a case of 47-year-old female patient with right eyelid swelling that progressed over a 12 year period and eventually underwent surgery with pathology showing AL conjunctival amyloidosis. Unlike in most other reported cases of localized amyloidosis, she was noted to have amyloid deposition in the bone marrow and gastrointestinal tract upon extensive evaluation without any evidence of underlying plasma cell dyscrasia. She has been on observation without evidence of systemic progression or recurrence of conjunctival amyloid.

Conclusions: Although it initially appeared that our case represented an isolated form of AL (kappa)-type conjunctival amyloidosis, systemic evaluation revealed trace amount of amyloid in the bone marrow and GI tract. It is feasible that upon very close scrutiny patients with seemingly localized AL amyloidosis may have trace amounts of amyloid involving other organs and based on experience from this single patient we believe that it is safe to observe such patients closely rather than pursue systemic therapy.

Keywords: Congo red; amyloidosis; multiple myeloma.

Figure 1

Right conjunctival amyloidosis.

Figure 2

Histological section of conjunctiva showing stromal nodular accumulation of proteinaceous acellular material with minimal associated perivascular plasma cells and lymphocytes. (hematoxylin and eosin, ×400).

Figure 3

Characteristic dichroism of amyloid on polarized light examination. (A) Congo red, ×200; (B) Congo red (polarized), ×200).