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 man presented with a history of lower back pain that had persisted for 2 years and difficulty walking for 2 months. The patient received a complete hemogram as well as routine biochemical and radiographic imaging studies. His hemoglobin level was 15.3 g/dL, his total leukocyte count was 6.1×109/L, his platelet count was 160×109/L, and his erythrocyte sedimentation rate (ESR) was 15 mm. No rouleaux formation was seen on a peripheral smear. Serum blood urea nitrogen (27 mg/dL), creatinine (1.06 mg/dL), calcium (9.9 mg/dL), and albumin (5.1 g/dL) were all within normal limits. Serum uric acid levels were high (7.6 mg/dL), Alkaline phosphatase levels were slightly increased (134 U/L), and the patient had subnormal vitamin D3 levels. Ultrasonography findings were within normal limits, except for mild prostatomegaly. Serum prostate specific antigen and carcinoembryogenic antigen levels were within normal limits.\nA radiograph of the dorsolumbar spine showed diffuse osteoporosis and wedging of the D12 vertebra. Multiple small permeative lesions were seen in the pelvic bones and proximal femoral shafts. MRI findings showed diffuse degenerative changes in the cervical and lumbar spine and D12 vertebral collapse. Bone scan showed increased uptake in the anterior region of the 6th rib, left sacroiliac joint, and occipital region. Serum protein electrophoresis did not show any M spike, and gamma globulin levels were normal (8.8 g/L). Urine protein electrophoresis showed protein levels of less than 60 mg/L and no M spike. The bone scan findings, together with increased serum uric acid and alkaline phosphatase levels, and negative myeloma workup suggested possible diffuse skeletal metastasis. The patient underwent bone marrow aspiration from the bilateral posterior iliac spines and unilateral trephine biopsy to look for any secondary causes of bone scan findings and to rule out the presence of a plasma cell neoplasm. The aspirates from both sides showed similar findings, with numerous plasma cells ranging from 60–70% (). Binucleated or multinucleated forms, plasmablasts, and occasional mitotic figures were also observed in those plasma cells. Additionally, intranuclear Dutcher bodies and occasional Mott cells were observed. The Congo red stain for amyloid on the trephine biopsy was negative. IHC was performed on the trephine biopsy, revealing cells positive for CD138, MUM 1, and cytoplasmic κ and negative for CD3, CD20, LCA, CD56, and lambda (λ). The Ki-67 labelling index was 15%. The mean λ FLC level was 11.2 mg/L (range, 5.5–26.3 mg/L), and that of κ was 176.8 mg/L (range, 3.3–19.4 mg/L). The mean κ/λ ratio was 15.78 (range, 0.2–1.65). Patient had high β2-microglobin levels, at 2,557 mg/mL (range, 609–2,366 mg/mL).\nThe patient was in clinical stage I according to the International Staging System (ISS) for plasma cell myeloma and stage IIIA according to the Durie-Salmon staging system. The patient was referred to the oncology center for further management. There the patient was treated with a chemotherapy regimen that included the proteasome inhibitor bortezomib and dexamethasone.