Multiple myeloma presenting as proliferative (crescentic) glomerulonephritis.
RA Sirsat, RB Deshpande
Dept of Nephrology, Hinduja National Hospital, Mahim, Bombay.
R A Sirsat
Dept of Nephrology, Hinduja National Hospital, Mahim, Bombay.
Glomerular extracapillary cellular proliferation with crescent formation initially presenting as rapidly progressive glomerulonephritis is a rare clinical manifestation of multiple myeloma. We report here a case of a 58 year old female who initially presented with haematuria, loss of weight and appetite and history of febrile episodes and was diagnosed following renal biopsy as rapidly progressive glomerulonephritis. Haemodialysis was carried out a month later because of uremic symptoms and maintained with monitoring of serum, calcium, phosphate, alkaline phosphatase, albumin and iPTH levels. After 6 months, she complained of bone pains over anterior chest wall which persisted even with low calcium haemodialysis. Serum protein electrophoresis and bone marrow aspiration revealed multiple myeloma. On starting chemotherapy, bone pain subsided but the patient expired within 15 days of therapy.
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Sirsat R A, Deshpande R B. Multiple myeloma presenting as proliferative (crescentic) glomerulonephritis. J Postgrad Med 1994;40:92-3
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Sirsat R A, Deshpande R B. Multiple myeloma presenting as proliferative (crescentic) glomerulonephritis. J Postgrad Med [serial online] 1994 [cited 2022 Jun 28 ];40:92-3
Available from: https://www.jpgmonline.com/text.asp?1994/40/2/92/553
Renal lesions specifically related to multiple myeloma are: a) cast nephropathy, b) amyloidosis and c) deposition of light chain fragments of immunoglobulins,. Uncommonly renal parenchyma may be infiltrated by atypical plasma cells. Other lesions associated with multiple myeloma are pyelonephritis, nephrocalcinosis and urate depostion.
Occasionally multiple myeloma may initially present as primary glomerular lesion such as mesangial sclerosis and mild thickening of the giomerular capillary basement membrane. In some cases these changes may be marked resembling mesangial proliferative glomerular nephritis.
Rarely there may be glomerular extracapillary cellular proliferation forming crescents, clinically presenting as rapidly progressive glomerulonephritis. We report here such a case.
A 58-year-old female presented in February 1992 with history of gross haematuria, loss of weight and appetite of six months duration; and fever off and on of six weeks duration.
Results of blood investigations were-as follows serum creatinine: 6.9 mg/dl; blood urea nitrogen 76 mg/dl: haemoglobin: 5.6 g/dl; serum calcium: 8.1 mg/dl; serum phosphate: 11.6 mg/dl; serum albumin: 3.5 g/dl. Her total white cell count was 12,500/cmm. Urine microscopy showed 90 red blood cells and 100 - 150 leucocytes per high field. Twenty-four hour urine protein was 1.5 gm. Blood culture grew Staphylococcus aureus on one occasion. Ultrasonography showed large echogenic kidneys and a stag horn calculus with minimal dilatation of pelvicalyceal system on the left side. Rest of the pelvis and abdomen were normal.
Percutaneous kidney biopsy showed total 22 glomerufl of which 10 were sclerosed to varying degree. The remaining glomeruli showed diffuse proliferation of endothelial cells with moderate infiltration of neutrophils in the glomerular tufts. There was moderate diffuse basement membrane thickening. In addition five glomeruli showed extracapillary cellular proliferation forming cellular and fibrous crescents [Figure 1].
There was focal tubular atrophy with interstitial infiltration by mononuclear cells. Congo red stain did not show amyloid deposition either on the glomerular capillaries, tubular basement membrane or the vessel walls.
Direct immunofluorescence studies using antibodies to IgG, IgM, IgA, C3 and fibrinogen were done. There was granular fluorescence along the glomerular capillary wall with antibodies to IgG and C3.
Two D echocardiogram did not show any vegetation on the valves. Even then, the patient was treated with penicillin.
A month later she was readmitted with uremic symptoms. She was put on maintenance haemodialysis.
Three months later in July 1992, the patient complained of bone pain over the anterior chest wall. Her serum calcium was 8.2 mg/dl: serum phosphate was 8.5 mg/dl and serum alkaline phosphatase was 80 units/I (normal range 40 - 120 units/L). Serum albumin was 2.9 g/dl serum iPTH level (mid region) was 2157 ng/dl.
The patient was treated with phosphate binders, calcium carbonate and calcithol. A month later her bone pain persisted. Serum calcium was 10.6 mg/dl: and serum phosphate was 3.10 mg/dl. Calcitriol and calcium were discontinued and the patient was started on low calcium haemodialysis.
Because of the persistence & bone pain, alternate explanation was sought. Serum protein electrophoresis showed increased gamma globulins with M band in IgG region. Serum protein immunofixation showed IgG kappa chain band. Bone marrow aspiration showed 35% atypical plasma cells confirming the diagnosis of multiple myeloma. Within a week of starting chemotherapy, her bone pain subsided. However. She expired suddenly 15 days after starting chemotherapy. Autopsy was not done.
The initial presentation of this case was that of rapidly progressing glomerulonephritis. There was no reason to suspect plasma cell dyserasia at that time. It was only six months later that she had bone pain. The bone pain was presumed to be a manifestation of renal osteodystrophy. She was treated with calcitriol, calcium supplements and phosphate binders, but with no improvement. It was only then that the possibility of myeloma was considered.
Glomerular proliferative lesions with crescent formation is rare in multiple myeloma,,,,.
In the case reported here immunolluorescence showed the presence of IgG and C3. This prompted us to consider the possibility of post-infectious glomerulonephntis. Blood culture on one occasion showed growth of Staphylococcus aureus. Possibility of endocarditis and other visceral source of infection were ruled out.
Occurence of crescentic glomerulonephritis multiple myeloma is explained in two ways: a) a post-infectious phenomena and b) secondary to deposition of light chain fragments of immunoglobulins along the capillary subendothelial region.
Initial presentation with fever, neutrophilic infiltration on the glomerular tufts, deposition of IgG and C3 along the capillary wall is consistent with the possibility of post-infectious process being the cause of crescentic glomerulonephtlis.
Immunofluorescence study of the present case did not include antibodies to light chain fragments of immunoglobulins. However, for reasons mentioned above, we feel the second possibility is less likely.
King JT, Venezuela R, McCormick U, Osborne DJ. Granular dense deposit disease. Lab Invest. 1978; 39: 591.|
|2||Randall RE, Williamson WC, Jr, Mullinax F, Tung MY, Still WJC. Manifestations of systemic light chain deposition. Am J Med 1976; 60:293-299.|
|3||Hill GS, Morel-Morger L, Mery JP, Browet JU, Mignon F. Renal lesions in multiple myeloma: Their relationship to the relative electrophoretic motility of urinary free light chains. Am J Kid Dis 1983; 2:423-438.|
|4||Silva FG, Meyrier A, Morel-Morger L, Pirani CL. Proliferative glomerulopathy in multiple myeloma. J Pathol 1980; 130:229-230.|
|5||Kaplan NG, Kaplan KC. Monoclonal gammopathy, glomerulonephritic and nephrotic syndrome. Arch Int, Med 1970; 125:696-700.|
|6||Dhar SK, Smith EC, Fresco R. Proliferative glomerulonephritis in monocional gammopathy. Nephron 1977; 19:288-294.|
|7||Meyner A, Simon P, Mignon F, Striker L, Ramee MP. Rapidly progressive ("crescentic") glomerulonephritis and monoclonal gammopathies. Nephron 1984; 38:156-162.|
|8||Rao TKS, Niscastri AD, Char CK, Rosenthal K, Friedman EA. Membranoprolif erative glomeruionephritis. An unusual manifestation of multiple myeloma (Abstract). Kidney Int 1978; 14:659.