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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2012  |  Volume : 23  |  Issue : 6  |  Page : 1285-1287
Primary amyloidosis treated with continuous ambulatory peritoneal dialysis

Department of Nephrology, Medwin Hospital, Nampally, Hyderabad, AP, India

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Date of Web Publication17-Nov-2012

How to cite this article:
Gude D, Chennemsetty S, Jha R, Narayan G. Primary amyloidosis treated with continuous ambulatory peritoneal dialysis. Saudi J Kidney Dis Transpl 2012;23:1285-7

How to cite this URL:
Gude D, Chennemsetty S, Jha R, Narayan G. Primary amyloidosis treated with continuous ambulatory peritoneal dialysis. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2023 Feb 6];23:1285-7. Available from: https://www.sjkdt.org/text.asp?2012/23/6/1285/103578
To the Editor,

Primary systemic amyloidosis (AL) is a plasma cell dyscrasia that is associated with considerable mortality and morbidity. It is characterized by the production and extracellular deposition of insoluble monoclonal immunoglobulin free light chain (FLC) fragments in organs and tissues. The age-adjusted incidence of AL is 5.1 to 12.8 cases per million person-years. [1] The prognosis, despite several advancements in treatment, continues to be poor, with a median survival in the range of 12 to 18 months. [2] Although hemodialysis (HD) and renal transplantation have established roles in the management of AL, there are very few studies documenting the efficacy of continuous ambulatory peritoneal dialysis (CAPD) as a therapeutic option in AL. We report a case of primary AL with end-stage renal failure who improved fairly well after being treated with CAPD.

A 42-year-old non-diabetic and normotensive female patient presented initially to the dermatologist with multiple hemorrhagic blisters over the hands, feet, trunk and face. A skin biopsy was performed, which showed features suggestive of pemphigus foliaceous erythematosus. Over the next decade, she presented with a confusing clinical picture with multisystem involvement. Clinical examination was characterized by edema of the feet with normotensive state and hyperpigmented skin lesions. Laboratory evaluation showed anti-nuclear antibody-negative nephrotic syndrome. A renal biopsy was performed, which was reported as minimal change disease. As there was no response to steroids, cyclophosphamide and cyclosporine, renal biopsy was repeated, which showed glomerular deposition disease due to AL. She was given angiotensin-converting enzyme inhibitors)/angiotensin II receptor blockers and statins for the nephrotic syndrome and topical steroids for blistering dermatosis. In 2005, she presented with low backache without any tenderness over the vertebrae. Detailed evaluation revealed monoclonal gammopathy (lambda chain/IgG), and blood examination showed hemoglobin (Hb) of 9 gm%, erythrocyte sedimentation rate (ESR) of 180, serum calcium of 8 mg/dL, phosphorous of 4.1 mg/dL and alkaline phosphatase (ALP) of 114 IU/L. Bone marrow biopsy was done, which did not show any plasmacytosis. The kidney biopsy was repeated, which showed amyloid deposits [Figure 1] that were potassium permanganate resistant, confirming the diagnosis of primary AL. Over the next few years, she had worsening dyspnea requiring multiple hospitalizations. 2D echocardiogram revealed moderate left ventricular dysfunction (LVD) (EF-34%) and speckled pattern of myocardium [Figure 2]. She also developed a swelling over the clavicular and thyroid regions, which was proved to be bony and thyroid amyloid. A final diagnosis of primary AL (monoclonal Ig deposition disease, restrictive cardiomyopathy/cardiac amyloid with LVD, blistering dermatosis with cutaneous amyloid and amyloid goiter) was made. She was started on HD but, because she had repeated vascular access failure, she was deemed unfit for long-term HD. Subsequently, she was started on CAPD with three exchanges, 2.5 L ultrafiltration and high average transporterstatus, and is doing well for the last six months without any further hospitalizations for congestive cardiac failure.
Figure 1: Kidney biopsy (400×) with Congo red stain depicting glomeruli with mesangial and vessel wall deposits (arrows) of acellular homogenous congophilic material (amyloid).

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Figure 2: Two-dimensional echocardiography (short axis view) showing speckled pattern in myocardium (arrow).

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Primary AL is characterized by multisystem involvement with renal, cardiac, neurologic and hepatic dysfunction inevitably leading to death. Cardiac AL especially is associated with a dismal prognosis, [3] although the presence of the nephrotic syndrome appears to be protective. A study quantified the incidence of various presentations in AL as follows: involuntary weight loss - 72%, hepatomegaly - 81%, proteinuria - 89% and elevated serum ALP levels - 86%. [4] The FLC assay is a quantitative and highly sensitive assay and helps monitor the disease activity more accurately.

In AL, there is a dynamic process of deposition and resolution of the precursor protein in the tissues that govern the disease severity. Hence, the active elimination of the precursor protein in organs might deter and even reverse the disease process, ultimately resulting in improved organ function and clinical performance. Regardless of the treatment modality, elimination of the precursor protein, thereby preventing further amyloid deposition in organs and eventually reaching resolution of amyloid, is the goal to be achieved. Chemotherapy has been the mainstay of treatment, and the rationale lies in targeting the clonal process and in checking the uninhibited formation of precursor protein. General tolerance to either chemotherapy or autologous stem cell transfusion (ASCT) is low, given the already jeopardized organ systems. Histological regression of amyloid in tissue has been documented after successful treatment with chemotherapy. A certain degree of reversibility in the disease pattern/severity can be expected with such tissue elimination of amyloid protein. In one study, complete response (complete normalization of elevated pre-treatment amyloidogenic serum FLC concentration, or, in case of complete renal failure, normalization of the ratio of kappa and lambda light chains) to treatment correlated well with superior survival. [5] Another study showed the reversible nature and hence the better prognosis of restrictive cardiomyopathy due to light-chain deposition, after remission of plasma cell dyscrasias. [6] The efficacy of adsorption of immunoglobulin light chains (Bence Jones proteins) on to polymethylmethacrylate membrane in a patient with primary AL was studied, and it was concluded that such techniques reliably result in clinical and biochemical improvement. [7] Immunoglobulin light chains, identical to the amyloid fibril (as determined by N-terminal amino acid analysis), have been demonstrated in the peritoneal fluid of a patient with AL [8] and, hence, the value of CAPD in AL can be inferred. Peritoneal dialysis as renal replacement therapy in patients with AL and/or paraproteinemia has shown promising results. The large peritoneal surface area helps remove immunoglobulins and light chains from the body. Such removal could prevent further amyloid formation, hyperviscosity syndromes and, in some cases, even reverse the renal insufficiency in these patients. [9] Studies have shown that the permeability characteristics of solutes (including immunoglobulins) across the peritoneal membrane in AL are unaffected, advocating CAPD as a valuable option in dealing with the disease. There are few studies documenting the efficacy of CAPD in AL, and we attempted to examine the efficacy of the same in our patient, with satisfactory results. Newer investigational therapies targeting the resorption of amyloid deposits include low-dose iodinated anthracycline [10] (produced organ response in 15% of AL patients) and passive anti-light chain immunization. [11]

We reiterate the efficacy of CAPD as an option in managing primary AL, especially if the patient is not fit for the standard chemotherapy and ASCT. We conclude that CAPD, as a stand-alone or adjunctive therapy in AL, definitely plays a role in bettering the patient's clinico-chemical profile, resulting in improved quality of life.

   Acknowledgment Top

The authors thank their colleagues and staff of the Department of Nephrology.

   References Top

1.Rachbauer F, Kreczy A, Bodner G. Amyloidoma of the clavicle. AJR Am J Roentgenol 2003;181:771-3.  Back to cited text no. 1
2.Dispenzieri A, Lacy MQ, Rajkumar SV, et al. Poor tolerance to high doses of thalidomide in patients with primary systemic amyloidosis. Amyloid 2003;10:257-61.  Back to cited text no. 2
3.Cueto-Garcia L, Reeder GS, Kyle RA, et al. Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival. J Am Coll Cardiol 1985; 6:737-43.  Back to cited text no. 3
4.Park MA, Mueller PS, Kyle RA, Larson DR, Plevak MF, Gertz MA. Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients. Medicine 2003;82:291-8.  Back to cited text no. 4
5.Skinner M, Sanchorawala V, Seldin DC, et al. High dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: An 8-year study. Ann Intern Med 2004;140:85-93.  Back to cited text no. 5
6.Nakamura M, Satoh M, Kowada S, et al. Reversible restrictive cardiomyopathy due to light-chain deposition disease. Mayo Clin Proc 2002;77:193-6.  Back to cited text no. 6
7.Hata H, Nishi K, Oshihara W, et al. Adsorption of Bence-Jones protein to polymethylmethacrylate membrane in primary amyloidosis. Amyloid 2009;16:108-10.  Back to cited text no. 7
8.Block PJ, Skinner M, Benson MD, Cohen AS. Identity of a peritoneal fluid immunoglobulin light chain and the amyloid fibril in primary amyloidosis. Arthritis Rheum 1976;19:755-9.  Back to cited text no. 8
9.Rosansky SJ, Richards FW. Use of peritoneal dialysis in the treatment of patients with renal failure and paraproteinemia. Am J Nephrol 1985;5:361-5.  Back to cited text no. 9
10.Gianni L, Bellotti V, Gianni AM, Merlini G. New drug therapy of amyloidoses: resorption of AL-type deposits with 4'-iodo-4'-deoxydoxorubicin. Blood 1995;86:855-61.  Back to cited text no. 10
11.Hrncic R, Wall J, Wolfenbarger DA, et al. Antibody-mediated resolution of light chain-associated amyloid deposits. Am J Pathol 2000;157: 1239-46.  Back to cited text no. 11

Correspondence Address:
Dilip Gude
Department of Nephrology, Medwin Hospital, Nampally, Hyderabad, AP
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.103578

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