A 74-year-old otherwise healthy woman presented with diffuse alopecia of the scalp. Two biopsies were taken for investigation of suspected telogen effluvium or diffuse alopecia areata. No clinical features of amyloidosis, such as macroglossia, purpura, infiltrative skin lesions or nail changes, were observed.\nThe skin biopsies showed no inflammation or evidence of a scarring alopecia. A large proportion of follicles were shifted into catagen and telogen phase in both biopsies. The residual fibrous stelae within the deep dermis and subcutis showed a remarkable appearance, with an empty center and a peripheral zone containing eosinophilic periodic acid Schiff (PAS)-positive and diastase-resistant ( and ), Congo-red-positive (), apple-green-birefringent material characteristic of amyloid. Similar amyloid deposits were also evident to a lesser extent surrounding follicular epithelium within the mid-dermis. There was no discernible amyloid deposition within dermal blood vessels, surrounding sweat apparati or adipocytes, or within the dermal interstitium.\nFurther special staining was carried out on one of the biopsies, with PAS with diastase (PASD) staining showing no fungal organisms or dermoepidermal basement-membrane-zone thickening. Alcian blue staining showed no dermal mucin accumulation and was negative within the amyloid deposits. Verhoeff staining showed no scarring that might suggest a scarring alopecia.\nWithin one biopsy, 14 of 37 (38%) hairs were vellus or vellus-like. Of the non-miniaturized hairs, 16 of 23 (70%) were in catagen or telogen phase. The other biopsy was more irregularly shaped and subsequently obliquely sectioned, allowing visualization of only 14 hairs, 5 of which (36%) were vellus or vellus-like; of the 9 remaining hairs, 2 were in catagen or telogen phase (22%).\nAs such, the biopsies were assessed to show a shift toward catagen and telogen hair follicles, consistent with the early phase of telogen effluvium. There was also moderate hair miniaturization consistent with pre-existing senile alopecia or androgenetic hair loss.\nPerifollicular deposition of amyloid prompted further workup of the patient for systemic forms of amyloidosis. Plasma protein electrophoresis and immunofixation showed a 1.4 g/L monoclonal band in the mid-to-late gamma region. Additional minor bands were noted in the beta-gamma region of both the alpha heavy chain and lambda light-chain classes and migrated with slightly different mobility, suggesting that they were not the same paraprotein. Urine protein electrophoresis showed moderate proteinuria in a glomerular pattern, without excretion of the monoclonal protein in the urine.\nBased on these findings, the patient was diagnosed with lambda AL amyloidosis. A bone-marrow biopsy performed 6 months after presentation showed a plasma-cell dyscrasia with lambda light-chain restriction and 8% plasma cells in the bone marrow. She subsequently received treatment with melphalan, and her disease is stable. At time of publication, she has no known involvement of the lymph nodes, oropharynx, liver, spleen, and cardiovascular system, and only mild renal impairment and proteinuria.
A 60-year-old male was referred to our clinic with 5-month history of gradual but progressive weight loss, recurrent fever, anorexia, progressive fatigue, and generalized bone pains. Two months after the onset of these symptoms, he developed inability to walk. There was no associated sphincteric dysfunction or sensory loss. There was no history of chronic cough. At the time of presentation, significant examination findings were pallor, inability to walk, and general bone tenderness. X rays of the skull, thoracolumbar spine, and pelvis showed lytic lesions (). Blood film and bone marrow aspiration were essentially normal with <5% plasma cells reported in the bone marrow. Erythrocyte sedimentation rate, C reactive protein, and serum protein electrophoresis were normal and urinary Bence Jones protein was negative. Laboratory data are summarized in .\nA bone marrow trephine biopsy was done and showed osteosclerotic changes and focal scalloping with prominent osteoclastic activity. The marrow was moderately to markedly hypercellular and was densely infiltrated by a cellular infiltrate. The cells were mostly small cells with fairly condensed chromatin. They showed a variable amount of cytoplasm and there was a fair sized subpopulation of plasmacytoid lymphocytes.\nImmunohistochemistry showed positivity of CD138 (which strongly supports the diagnosis of plasma cell myeloma) and cyclin D1 (which is associated with cases of plasma cell myeloma with lymphoplasmacytic morphology and correlates with the presence of the translocation t(11;14)(q13;q32)).