allopurinol and Paraproteinemias

The technique of crossed immunoelectrophoresis (X-IEP) has been used to quantify free monoclonal light chains (LC) directly in the serum of patients with light chain disease, without preliminary gel or membrane filtration of serum to separate whole immunoglobulin. LC concentration is proportional to the area under an immunoprecipitin peak formed by LC in the patient's serum and an anti-LC antibody of appropriate specificity. Light chains of beta electrophoretic mobility can be processed in the standard X-IEP technique at pH 8.6. Light chains of gamma mobility must be processed in a modified technique using a pH of 5.0 and a carbamylated antiserum in the second dimension gel. Dose response curves obtained from the method in serial dilution experiments with sera from 18 patients gave correlation coefficients greater than or equal to 0.99. Replicate measurements of absolute LC concentration on the same specimens were within +/- 10%. The method can also detect polymerized light chains. Serum light chain levels were measured during the course of plasmapheresis therapy in three patients with light chain disease and renal failure. Light chain levels were shown to fall after plasmapheresis and to rise rather rapidly in the interval between treatments.

Topics: Acute Kidney Injury; Aged; Allopurinol; Calcitonin; Carmustine; Counterimmunoelectrophoresis; Cyclophosphamide; Drug Therapy, Combination; Female; Humans; Immunoglobulin lambda-Chains; Immunoglobulin Light Chains; Male; Melphalan; Paraproteinemias; Peritoneal Dialysis; Plasmapheresis; Prednisone; Vincristine

A 33-year-old man with overall renal function in the lower normal range had daily excretion in the urine of between 31 and 70 gm of protein composed entirely of free monoclonal K light chains. K light chains were also present in the serum. Serum protein electrophoresis and findings on bone marrow and lymph node biopsy were diagnostic of light chain disease. Amyloid was absent from renal tissue. General clinical improvement and almost total disappearance of protein from the urine followed treatment with phenylalanine mustard.

Topics: Adult; Allopurinol; Humans; Immunoglobulin kappa-Chains; Immunoglobulin Light Chains; Male; Melphalan; Paraproteinemias; Proteinuria

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.

Topics: Allopurinol; Anemia; B-Lymphocytes; Cryoglobulins; Female; Humans; Leukopenia; Lymphatic Diseases; Melphalan; Middle Aged; Paraproteinemias; Prednisone; Purpura, Hyperglobulinemic