DETECTION, SIGNIFICANCE AND TREATMENT OF PARAPROTEIN IN PATIENTS PRESENTING WITH IDIOPATHIC PROTEINURIA WITHOUT MYELOMA

  • 1 January 1978
    • journal article
    • research article
    • Vol. 47  (186) , 145-175
Abstract
The detection of a paraprotein in blood or urine in 12 of 260 patients with idiopathic proteinuria, most of whom presented with the nephrotic syndrome, was described. None had myeloma at presentation and only 2 later developed it. Initial clinical and biochemical findings did not suggest paraprotein-associated disease; total serum globulins and individual immunoglobulin levels were usually in the normal range. In 7 of the 12 cases the paraprotein was detected only after repeated analysis of serum and urine specimens over months or years. Renal histopathology varied from case to case and is described in detail; amyloid deposition did not occur in patients who excreted .kappa. chain Bence Jones protein and was extensive in only 3. Of these, 1 eventually developed myeloma. Patients were aged 27-69 yr at onset and were observed without specific therapy for up to 56 mo. Glomerular filtration rate tended to decline and proteinuria persisted. All patients were treated by a chemotherapeutic regimen consisting of 1,3-bis(2 chloroethyl)-1-nitrosourea, cyclophosphamide, melphalan and prednisolone, in repeated short courses. In some patients, particularly those who had .kappa. Bence Jones protein, there was striking improvement. Overall survival was good, 8 patients being alive 17-90 months after the onset of symptoms. The importance of repeated search for paraprotein in apparently idiopathic renal disease in adults is emphasized.

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