So-called primary systemic amyloidosis probably due to myelomatosis.
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The authors review 18 patients with multiple myeloma who had bone destruction of a kind that indicated surgical therapy. Eight patients had paralegic myelopathy and one had compression of the cauda equina. Four of them displayed partial to complete regression. One patient lived for 77 months after the operation, most of the time in excellent condition. The operative technique is discussed, with laminectomy, exeresis, filling of bone with cement and, in some instances, mechanical support from metal plates. Early diagnosis and operation is imperative, postoperative irradiation obligatory in severe cases. Radiation alone may be the method of choice in early stages. The other 9 patients were operated upon for bone destruction in the limbs. A Moore operation on the destroyed hip was performed in one patient, who lived in excellent condition for about four years. Active surgical therapy combined with radiation and cytostatics seems to be of value in many patients with multiple myeloma.
A case of primary amyloidosis is described in association with Bence-Jones myeloma and increased fibrinolytic activity. Epsilon aminocaproic acid reversed the fibrinolytic activity and clinical improvement was maintained with melphalan and prednisolone.
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Two cases of multiple myeloma with IgA gammopathy and the syndrome of serum hyperviscosity are reported. A common feature of both patients was the production of large amounts of polymeric IgA (more than 50% of the monoclonal proteins existed as dimers or larger forms). However, similar large amounts of polymeric IgA were present in sera with only slightly raised viscosity, suggesting that factors such as the molecular configuration of the polymers or their sub-units may play a role in determining serum viscosity. The association of plasmapheresis and chemotherapy produced remarkable improvements of the symptoms attributable to increased serum viscosity, and 16 months after diagnosis one of the patients has survived relatively free of symptoms. The hyperviscosity syndrome is being diagnosed with increasing frequency in association with IgA multiple myeloma, and should be investigated in all cases with monoclonal proteins exceeding a concentration of 3-0 g/dl.
Factor VIII and factor-VIII-related antigen (FVIIIRA) were estimated in 19 patients with paraproteinaemia. The FVIIIRA levels were above the normal range in 12 cases with II of these showing an abnormal FVIII/FVIIIRA ratio. The patients could be subdivied into three groups according to their FVIIIRA levels: normal levels, levels greater than normal but less than 200%, greater than 200%. This subdivision correlated with the degree of bone involvement as assessed by radiological skeletal survey.
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A case of systemic light chain deposition disease in a 48-year-old man is presented. Clinically, the patient showed the signs of multiple organ (liver, heart and kidney) failure, but multiple myeloma was not diagnosed. Autopsy revealed generalized deposition of hyaline, a Congo red-negative substance, especially in the arterial walls of various organs. In the bone marrow, myelomatous proliferations of plasma cells positive for kappa light chain were recognized. The deposited substance was ultrastructurally different from amyloid fibrils, and was identified as kappa light chain by immunohistochemistry. A liver tissue extract was immunobiochemically examined and the deposited substance was confirmed to consist of kappa light chain, its molecular weight being approximately 14,000 to 17,000 Da.