Atypical amyloid deposits

Excerpt

A 57-year-old man presented with a 6-month history of fatigue, fever, polyarthralgia, dysphagia, paresthesia of legs and feet with significant weight loss. At physical examination, bilateral endured hyperchromic plaques on periorbital region (Fig. 1) and an atypical enlargement of the tongue with deep excavations on lateral and anterior borders (Fig. 2) were observed. Echocardiogram showed both systolic and diastolic disfunction and an ejection fraction of 37 %. Bone marrow aspirate contained 22 % of monoclonal plasma cells. A bone marrow biopsy was performed, and immunohistochemical studies showed plasma cells positive for lambda light chain and CD138, and negative for kappa (Fig. 3a–c). Congo-red staining of the tongue and periorbital skin (Fig. 4) showed accumulation of salmon coloring in dermis, evidencing amyloid deposition. A diagnosis of lambda light chain multiple myeloma with systemic amyloidosis was established. The patient was enrolled in chemotherapy protocol bortezomib, cyclophosphamide and dexamethasone with important regression of the skin and tongue lesions (Fig. 5).

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