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 complaints of low back ache of one year duration. The pain was radiating down the right lower limb as far as the knee joint. There was no history of trauma or history suggestive of any significant medical co-morbid conditions.\nExamination revealed tenderness on the right side of the lumbosacral spine and right sacroiliac joint. There was para vertebral muscle spasm with loss of lumbar lordosis. Hip movements were normal with no major disability. There were no root stretch signs and no neurological deficit was noted.\nAn x-ray of the lumbosacral spine showed evidence of old fracture of L1 vertebra []. The Magnetic Resonance Imaging (MRI) scan showed collapse of the L2 vertebra with extrusion of the bone posteriorly into the spinal canal []. There was soft tissue infiltration seen in the sacrum. While awaiting clinic review the patient presented to the casualty with fracture clavicle on the left side following a trivial fall. The fracture of the clavicle was at the medial end (unusual site). A subsequent bone scan showed increased uptake in the medial end of the left clavicle and L2 vertebra. There were areas of increased activity in the region of the ribs [].\nLab investigations revealed hemoglobin of 12.8 g/dl, erythrocyte sedimentation rate (ESR) of 21mm/hr and hematocrit ratio of 0.357 (normal 0.370-0.470 ratio). Creatinine was 50 μmol/l (normal 50-141μmol/l) and urea of 5.9mmol/l (normal 2.5-7.5 mmol/l). The serum calcium 2.28 mmol/l (normal 2.1-2.6 mmol/l) and phosphate levels 1.13 mmol/l (normal 0.81-1.45 mmol/l) were normal. Lactic acid dehydrogenase was elevated, 1061 IU/l (normal 313-618 IU/l) and Alkaline phosphatase was normal, 125 IU/l (normal 25-150 IU/l).\nMyeloma screen which did not reveal presence of any abnormal compact bands was obtained and there was no Bence-Jones proteinuria. The levels of the immunoglobulin were Ig G 6.6 g/l (normal 5.3-16.5 g/l), Ig A 0.21 g/l (normal 0.80-4.00 g/l) and Ig M was 0.36 g/l (normal 0.50-2.00 g/l), which were lower than normal.\nIn view of the bony pain, pathological fracture of the L2 vertebra, unusual fracture of the clavicle, normal myeloma screen, normal calcium levels and bone scan findings it was decided to organize a Computerized Tomography (CT) scan of the thorax and abdomen to look for the primary with secondaries as the first working diagnosis. The CT scan merely confirmed the findings of MRI scan and there was no evidence of any primary or other secondaries []. A bone biopsy was planned.\nA percutaneous bone biopsy from the medial end of the clavicle was done because of easy accessibility which was negative for malignancy. A CT guided biopsy from the L2 vertebra was again negative for malignancy. A bone marrow aspirate and trephine biopsy were done from the iliac crest which confirmed the diagnosis of multiple myeloma. The biopsy showed a hyper cellular marrow with normal haemopoiesis replaced entirely with malignant plasma cells suggestive of myeloma. Aspirate showed plasma cells to be about 52% of the marrow with marked atypia. The patient was referred to further treatment in the form of chemotherapy and radiotherapy. The patient died one month after diagnosis while undergoing treatment. There was, however, a delay of two months between the first presentation and final diagnosis.