ABSTRACT
UNLABELLED: IgD myeloma, which is particularly severe, accounts for only 1-3% of all myeloma cases, and the kappa subtype contributes only 10-30% of IgD myelomas. We report a new case. CASE-REPORT: A 59-year-old man was admitted for inflammatory low back pain with L5 sciatica and diffuse bone pain. The symptoms had been present for 3 months, during which he had experienced a severe decline in general health. Laboratory test abnormalities included an erythrocyte sedimentation rate of 70 mm/h, normochromic normocytic aplastic anemia, hypercalcemia (3.5 mmol/l), and serum creatinine elevation to 583 micromol/l. Tests were negative for cryoglobulinemia. Serum protein electrophoresis showed hypergammaglobulinemia but no monoclonal peak. Immunoelectrophoresis, however, detected a faint IgD kappa band in the blood and a homogeneous kappa band in the urine. Bone marrow aspirated from the sternum was found to contain 30% of malignant plasma cells. Biopsies for amyloidosis were negative. Radiographs disclosed multiple punched-out lesions with no evidence of spinal cord compression. Symptomatic treatment was given to correct the hypercalcemia, and combination chemotherapy was started. DISCUSSION: IgD kappa myeloma is a severe variant of myeloma often associated with extraosseous lesions, renal failure, and amyloidosis. The monoclonal component is absent or faint by serum protein electrophoresis, making the diagnosis difficult. The pathogenesis is unclear and the prognosis grim.
Subject(s)
Immunoglobulin D , Immunoglobulin kappa-Chains , Multiple Myeloma/immunology , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Chlorambucil/administration & dosage , Humans , Hypercalcemia/etiology , Hypercalcemia/pathology , Immunoglobulin D/blood , Immunoglobulin D/urine , Immunoglobulin kappa-Chains/blood , Immunoglobulin kappa-Chains/urine , Male , Middle Aged , Mitoxantrone/administration & dosage , Multiple Myeloma/drug therapy , Multiple Myeloma/metabolism , Prednisolone/administration & dosage , Renal Insufficiency/etiology , Renal Insufficiency/pathology , Treatment OutcomeABSTRACT
INTRODUCTION: Primary hypoparathyroidism and adhesive capsulitis of the shoulder in the same patient does not seem to have been reported previously. We report two cases in first-degree relatives. PATIENTS: Case 1: In 1999, a 41-year-old woman experienced seizures simulating epilepsy. She had been treated 5 years earlier for idiopathic adhesive capsulitis of both shoulders. Computed tomography of the brain showed calcifications in the basal ganglia meeting criteria for Fahr's disease, and incipient bilateral cataract was found. A diagnosis of idiopathic primary hypoparathyroidism was given. After 1 month of calcium and vitamin D supplementation, improvements were noted in the clinical symptoms, laboratory test, and electroencephalogram. Case 2: The 70-year-old father of case 1, who had an unremarkable medical history, had been receiving treatment for about 2 years for adhesive capsulitis of the left shoulder. Routine laboratory tests disclosed idiopathic primary hypoparathyroidism. The outcome was favorable with calcium and vitamin D supplementation. CONCLUSION: Our cases suggest that there may be a common immunological or genetic basis for primary hypoparathyroidism and adhesive capsulitis. Alternatively, adhesive capsulitis may be a manifestation of hypoparathyroidism. Although genetic factors involved in primary hypoparathyroidism have been elucidated, the pathophysiology of the disease remains unclear. Finally, a chance association remains possible.
