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In lymphoplasmacytic lymphoma (LPL) also known as
Waldenstrom’s macroglobulinaemia (WM) renal disease is less common than in
multiple myeloma. WM accounts for about 5% of all malignant B-cell disorders
associated with a monoclonal protein spike in the serum or urine and about half
of patients who have a monoclonal Immunoglobulin M (IgM) spike in the serum
have this disorder. Plasma cell dyscrasias often are associated with kidney
diseases due to the production of monoclonal immunoglobulin but with a diverse
set of pathologic renal patterns. While many patients undergoing a renal biopsy
and showing a cast nephropathy have multiple myeloma (MM), kidney involvement
associated with pathological immunoglobulin light chains and lymphoma is rare.
Thus a therapeutic approach that decreases light chain production appears to be
warranted in such patients.
Keywords: Waldenstrom’s macroglobulinaemia, Lymphoma, Nephropathy
INTRODUCTION
Multiple
myeloma is very commonly associated with renal failure. A renal biopsy in such
patients shows a cast nephropathy but the association of cast nephropathy with
lymphoma is quite rare [1]. Some of these patients develop salt losing
nephropathy as a renal complication in lymphoplasmacytic lymphoma and then
subsequently there is a diagnostic dilemma in distinguishing the causes for
renal dysfunction. The incidence of renal complication in waldenstrom's
macroglobulinemia (WM) is very less compared to the incidence in those of
myeloma patients. This is probably due to the rare occurrence of Bence-Jones
proteinuria in WM which may also explain why tubular casts and so called
‘myeloma kidney’ are rare in WM [2]. In WM renal disease is usually caused by
IgM deposits along the glomerular basement membrane, infiltration of the
interstitium with lymphoid cells or amyloidosis [3].
DISCUSSION
We have had an experience of few patients who
presented with renal failure with a slight proteinuria with kappa and lambda
light chains on immunoelectrophoresis. Renal involvement in WM presents as
usually a mild non-selective proteinuria and microscopic hematuria. Massive
proteinuria and a nephrotic syndrome may develop and in most cases is caused by
amyloidosis [3]. Bence Jones proteinuria is present in 80-90% of the patients,
but the quantity is much smaller than in MM. Cryoglobulinemia and acute renal
failure are rare in WM. In most cases renal failure is chronic, but due to
inadequacy of follow-up data, not much is known about the course of renal
failure in WM [3,4]. The two major classes of light-chain proteins are kappa
and lambda, which are being synthesized in bone marrow plasma cells. Each major
type can be further classified by the use of appropriate antisera into several
subtypes, four kappas and five lambdas [5].
Kidney is the major site of metabolism of light-chain proteins. Even though the complete Ig (molecular weight 160,000 to 900,000) and heavy chains do not pass through the normal glomerular filtration barriers, the small light chains can freely pass through. These filtered proteins, reabsorbed by proximal tubular cells are then catabolized by lysozymal enzymes. Normally, this highly efficient process leaves only a minute amount of light-chain protein, which then appears in the urine. Thus, metabolism depends on the function of the proximal tubular cell and damage to these cells can result in increased excretion of light-chain proteins in the urine. In diseases in which the production of light-chain proteins is markedly increased, the ability of the proximal tubules to reabsorb all the filtered protein is exceeded and light-chain proteins appear in the urine in high concentrations in this setting as well [4].
Light-chain proteinuria is common in WM; its
prevalence ranging from 30% to 40%. Patients with WM have a plasma cell
dyscrasia and they produce IgM paraproteins. As IgM has a tendency to form a
pentamer, patients with the disease are much more likely to develop high serum
viscosity than are patients with MM. The overall prevalence of Nephrotic
Syndrome (NS) in Hodgkin disease is about 0.4% and is even lower in non-Hodgkin
lymphomas (NHLs) [4]. Both idiopathic and malignancy-associated NS are thought
to be mediated by a soluble permeability factor, still unidentified, which
causes loss of selective capillary permeability and allows albumin and other
negatively-charged molecules to cross the glomerular barrier. In
lymphoma-associated NS, the permeability factor is supposed to be
paraneoplastic in origin [4,5].
CONCLUSION
Lymphoma-associated kidney involvement occurs
by a variety of mechanisms, which differ widely in prevalence and clinical
presentation. One of our patient with WM and Salt losing nephropathy caused by
IgM deposits, who subsequently treated with chemotherapy and resulted in an
improvement to a normal renal function and disappearance of proteinuria.
1. Lindström
FD, Hed J, Eneström S (1980) Renal pathology of Waldenstrom’s macroglobulinemia
with monoclonal anti-glomerular antibodies and nephrotic syndrome. Clin Exp
Immunol 41: 196-204.
2. Veltman
GA, Van Veen S, Nelemans JCK, Bruijn JA, Van Es LA (1997) Renal disease in
Waldenstrom’s macroglobulinemia. Nephrol Dial Transplant 12: 1256-1259.
3. Tubbs
RR, Gephardt GN, McMahon JT, Hall PM, Valenzuela R, et al. (1981) Light-chain
nephropathy. Kidney Am J Med 71: 263-269.
4. Cohen
LJ, Rennke HG, Laubach JP, Humphreys BD (2010) The spectrum of kidney
involvement in lymphoma: A case report and review of the literature. Am J
Kidney Dis 56: 1191-1196.
5.
Pérez NS,
Herrera AG, Rosinol L, Palos L, Santiago E, et al. (2012) Lymphoplasmacytic
lymphoma causing light chain cast nephropathy. Nephrol Dial Transplant 27:
450-453.
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