We present a case of a 68-year-old white female who presented with the complaint of fatigue and hair loss for several months. She had a past medical history of benign essential hypertension, bradycardia s/p (status post) cardiac pacemaker secondary to Mobitz type 2 atrioventricular block, hyperlipidemia, essential tremor, eczema, and osteopenia. An evaluation by dermatology had confirmed a diagnosis of alopecia totalis. This progressed to complete hair loss over the next few months. She was referred to nephrology for worsening creatinine (0.9-1.9 mg/dL over the course of 1 year) and proteinuria. On physical examination, she exhibited normal vital signs. Skin examination revealed complete hair loss of the body including the scalp (), eyebrows, eyelashes, and axillary () and pubic hair. Cardiac and lung examinations were unremarkable although she was noted to have trace of lower extremity edema.\nUrinalysis disclosed significant albuminuria, albumin/creatinine ratio of 8 g/g of creatinine, and urine protein/creatinine ratio of 9.8. Serum albumin was low at 3 g/dL. Urine microscopic examination was bland. Serological workup including anti-Ro, anti-La, anti-RNP, anti-Sm, anti Scl-70, anti Jo-1, ANA, anti–double stranded DNA, ANCA screen, C3, C4, hepatitis profile, HIV screen, cryoglobulins, and rheumatoid factor were all negative. Serum and urine protein electrophoresis revealed no paraproteins. She was noted to be anemic with hemoglobin of 12 g/dL. Given declining renal function, significant proteinuria, and a negative workup, renal biopsy was pursued. This revealed eosinophilic material in the mesangium with apple-green birefringence on Congo-red stain. Deposition of fibrillary material was seen in electron microscopy (). Immunohistochemistry of the biopsy specimen confirmed the presence of amyloid, AL type (kappa). Serum-free light chain analysis demonstrated an elevated kappa/lambda ratio of 79.8; kappa concentration was 1014 mg/dL, while free lambda was 12.7 mg/dL. Bone marrow examination showed 28% plasma cells with atypia 46, XX; fluorescence in situ hybridization demonstrated monosomy-13 with gains in chromosomes 9 and 11.\nThe patient was initiated on bortezomib and dexamethasone treatment for amyloidosis associated with MM. She was hospitalized 1 month later for symptoms of heart failure and was found to have a reduced left ventricular ejection fraction of 25%. Cardiac dysfunction was presumed to be secondary to amyloidosis. Her renal failure continued to progress and required initiation of hemodialysis. She was switched to ixazomib because of severe adverse effects to bortezomib after 3 cycles. She had a very good partial response to chemotherapy with serum kappa light chain level decreasing from 1854 mg/dL to 165 mg/dL. Renal function failed to recover and she remained dialysis dependent. She experienced an improvement in alopecia with new hair growth following the initiation of chemotherapy coincident with a significant reduction in the kappa light chain levels. The favorable response to chemotherapy may be predictive of an improved overall prognosis in our patient.
The present case is of a 60 year old woman who initially presented with back pain. There was no history of trauma or history suggestive of any comorbid conditions.\nExamination revealed tenderness on right side of lumbosacral spine and right sacroilliac joint. Hip movements were normal.\nMRI scan showed wedge compression fracture of D6, D7, D9 & D10 vertebrae with areas of altered signal intensity involving right sided pedicle of D9 & D10 vertebrae compressing the thecal sac and dorsal spinal cord.\nEdema in dorsal spinal cord at D10 level was also noted.\nLab investigations revealed\nHemoglobin of 13g/dl,\nTotal Leukocyte Count of 10,890 and\nPlatelet count of 1.5 lac/cumm.\nESR =27mm/hr\nViral markers revealed HCV positive (incidental finding).\nHypercalcemia with a serum calcium level of 12.5g/dl.\nPBF shows normal blood picture with mild neutrophilia (75%) and no rouleaux formation.\nBone marrow aspiration and biopsy revealed plasmacytosis (>45%)\nSerum protein electrophoresis showed absence of M band with hypogammaglobulinemia.\nThe above patient meets the criteria laid down by the international myeloma working group and chronic leukemia myeloma task force.\nIt was also a diagnostic dilemma as other disorders such as secondary deposits, osteoporosis and hyperparathyroidism can present with a similar picture.\nOur patient did not undergo serum immunoglobulinfree light chain assay (FLC).\nNow patient is currently being managed on the lines of treatment of multiple myeloma.