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Year : 2020  |  Volume : 63  |  Issue : 3  |  Page : 509-510
Multiple myeloma with secondary light chain deposition disease


Department of Pathology, Karnataka Institute of Medical Sciences, Hubballi, Karnataka, India

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Date of Submission10-Jul-2018
Date of Decision25-Sep-2018
Date of Acceptance09-Oct-2018
Date of Web Publication7-Aug-2020
 

How to cite this article:
Giriyan SS, Reddy P, Sekar MD. Multiple myeloma with secondary light chain deposition disease. Indian J Pathol Microbiol 2020;63:509-10

How to cite this URL:
Giriyan SS, Reddy P, Sekar MD. Multiple myeloma with secondary light chain deposition disease. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Oct 25];63:509-10. Available from: https://www.ijpmonline.org/text.asp?2020/63/3/509/291732




Dear Editor,

Light chain deposition disease (LCDD) is characterised by the accumulation of monoclonal light chains in many organs, including the kidney, skin, heart, liver, lungs and gastrointestinal tract.[1] The light chains may be kappa or lambda, but are usually kappa. Renal involvement is a constant feature of LCDD; it is the major manifestation in most patients.

A 78-year-old woman, known hypertensive, presented with oliguria and pedal oedema since 20 days. Her investigations revealed anaemia with a haemoglobin count of 8.8 g/dl, an elevated blood urea of 59 mg/dl and creatinine of 3.3 mg/dl. Serum calcium level was 8.3 mg/dl. Her lipid profile was normal. Total protein was 3.9 g/dl, albumin was 2.4 g/dl and her 24 h urinary protein was 4.8 g/day. A clinical diagnosis of hypertension with chronic kidney disease was made and renal biopsy was done in view of severe proteinuria. Renal biopsy was reported as glomeruli showing lobular accentuation with periodic acid-Schiff (PAS) positive nodular matrix-forming mesangial nodules. There was mild-to-moderate cellularity in the mesangium. One of the glomeruli showed circumferential active cellular crescent and there was no double contour appearance of basement membrane. Tubules showed diffuse presence of thickened basement membrane. There were no PAS positive cast in the lumen or any giant cell reactions to cast. Tubular atrophy/dropout was noted approximately in 25–30% of the cortical areas. Congo red stain for amyloid was negative. In immunofluorescence no glomeruli were seen in renal tissue. Thickened tubular basement membrane showed kappa (4+) light chain restriction over negative lambda light chain. Granular deposits were not seen with panel of antisera (IgG, IgM, IgA, C3 and C1q). Hence the diagnosis of native – LCDD (kappa light chain restricted) with solitary crescent and moderate tubulointerstitial chronicity was given. Following which bone marrow examination, serum/urine immunofixation and free light chain assay were considered. Her serum protein electrophoresis revealed a sharp increase in α2 globulin region (30.2%) suggesting nephrotic syndrome/monoclonal component. Serum/urine protein immunofixation and free light chain assay could not be done. X-ray skull and pelvis showed punched out lytic lesions in the skull. Bone marrow aspiration showed hypercellular marrow and there was an increase in plasma cells which constituted 30% of marrow cells [Figure 1]. Few atypical plasma cells were seen. On considering the presence of one major and one minor criteria, diagnosis of multiple myeloma was made on bone marrow. Thus the diagnosis of LCDD associated with multiple myeloma was made.
Figure 1: Bone marrow aspiration image showing mature plasma cells along with erythroid and myeloid series cells. Leishman stain 100×

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LCDD occurs due to overproduction and extracellular deposition of monoclonal immunoglobulin (Ig) light chain. Most commonly patients present with proteinuria or renal insufficiency causing nephrotic syndrome. As in our case, serum protein electrophoresis showed a sharp increase in α2 globulin region which might be seen in conditions such as adrenal insufficiency, adrenocorticosteroid therapy, advanced diabetes mellitus, nephrotic syndrome or it can be a monoclonal component.[2] Very rarely M-protein that is seen in α2 globulin region is reported in a few number of IgA multiple myeloma cases.[3]

The most characteristic lesion is nodular glomerulosclerosis, resembling diabetic glomerular disease, is present in 60% of cases.[4] The glomeruli are enlarged, with accentuated lobulation of the tuft and nodular expansion of the mesangium by multiple, acellular, PAS-positive, non-argyrophilic and Congo red-negative material. The glomerular capillary walls are often thickened by similar material and sometimes there might be small segments of mesangial cell interposition with reduplication of the basement membrane. Mesangial cell proliferation is slight or absent.[5]

The tubules often show some atrophy and there might be a bright, PAS-positive, Congo red-negative, refractile and ribbon-like thickening of their basement membranes on the outer side, and this finding is most prominent in the medulla. The tubular basement membrane deposits are smooth and linear by fluorescence microscopy.[5]

Nodular glomerulosclerosis of LCDD should be differentiated from renal amyloid (nodules positive with Congo red stain), nodular diabetic glomerulosclerosis (hyalinisation of afferent and efferent arteriolar walls) and diffuse mesangiocapillary glomerulonephritis (extensive mesangial interposition present).

The exact mechanism of tissue deposition of light chains is still not known. However, many suggestions are available which includes major structural abnormalities that favour the deposition of Ig chains. Glycosylation of the Ig molecules and their trend to assemble into high-molecular-weight polymers might play a role in their deposition. Normal-sized Ig may bear other or minor abnormality that lead to their deposition. Isoelectric (pI) point of light chains or their possible affinity for biological membrane might also play a role and polymerisation of the light chains when associated with a low pI would further predispose for renal deposition.[5]

Within a few years, most of the patients progress to end-stage renal disease. Symptomatic management may have a favourable effect on renal function independent of chemotherapy.[5] Renal transplantation has shown good results, but over years light chains gradually deposits in the graft, however, death usually occurs due to the complications of underlying lymphoid or plasmacytic proliferative disorder.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Randall RE, Williamson WC, Mullinax F, Tung MY, Still WJS. Manifestations of systemic light chain deposition. Am J Med 1976;60:293-9.  Back to cited text no. 1
    
2.
Kyle RA. Sequence of testing for monoclonal gammopathies. Arch Pathol Lab Med 1999;123:114-8.  Back to cited text no. 2
    
3.
Nargis W, Ibrahim M. A case of multiple myeloma with unusual serum protein electrophoresis. Pulse 2015;8:77-80.  Back to cited text no. 3
    
4.
Lin J, Markowitz GS, Valeri AM, Kambham N, Sherman WH, Appel GB, et al. Renal monoclonal immunoglobulin deposition disease: The disease spectrum. J Am Soc Nephrol 2001;12:1482-92.  Back to cited text no. 4
    
5.
Ganeval D, Noel LH, Preud'homme JL, Droz D, Grunfeld JP. Light-chain deposition disease: Its relation with AL type amyloidosis. Kidney Int 1984;26:1-9.  Back to cited text no. 5
    

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Correspondence Address:
Mithraa D Sekar
Department of Pathology, Karnataka Institute of Medical Sciences, PB Road, Vidyanagar, Hubballi - 580 021, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_429_18

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