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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 3  |  Page : 419-421
Non-Hodgkin's Lymphoma in Autosomal Dominant Polycystic Kidney Disease, 12 Years after Renal Transplantation


Department of Kidney Transplant and Pathology, Ekbatan Hospital, Hamadan Medical University, Hamadan, Iran

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   Abstract 

Autosomal dominant polycystic kidney disease (ADPKD) is a systematic disease which accounts for 10-15% of patients receiving dialysis or renal transplantation. It has a statistically significant association with malignancy in renal transplant recipients. We report a 47­year-old ADPKD female who developed a large renal tumor in the right kidney 12 years after kidney transplantation. During the follow-up, her ultrasound and laboratory tests were within normal limits. Bilateral nephrectomy of the native kidneys was performed, and followed by radiotherapy on the right side because pathology of the tumor suggested Non-Hodgkin's Lymphoma (NHL).

Keywords: Non-Hodgkin′s, Lymphoma, Renal, Carcinoma, Autosomal, Dominant Polycystic, Kidney, Disease, Transplantation

How to cite this article:
Amirzargar MA, Dadras F, Khoshjoo F, Yavangi M, Jafary M, Bahar SH, Amirzargar N. Non-Hodgkin's Lymphoma in Autosomal Dominant Polycystic Kidney Disease, 12 Years after Renal Transplantation. Saudi J Kidney Dis Transpl 2007;18:419-21

How to cite this URL:
Amirzargar MA, Dadras F, Khoshjoo F, Yavangi M, Jafary M, Bahar SH, Amirzargar N. Non-Hodgkin's Lymphoma in Autosomal Dominant Polycystic Kidney Disease, 12 Years after Renal Transplantation. Saudi J Kidney Dis Transpl [serial online] 2007 [cited 2020 May 26];18:419-21. Available from: http://www.sjkdt.org/text.asp?2007/18/3/419/33762

   Introduction Top


Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common hereditary disorders. [1],[2] It affects approximately 4 to 6 million individuals worldwide.[1],[3] ADPKD ultimately progresses to End-Stage Renal Disease (ESRD), and accounts for 10­15% of patients requiring dialysis or kidney transplantation. [4],[5] Renal polycystic disease is the only cause of renal failure that has a statistically significant association with the acquisition of a malignancy in renal transplant recipients. [4],[6] According to a review of the literature this is the first case of Non­Hodgkin's Lymphoma (NHL) in ADPKD after renal transplantation.


   Case Report Top


A 47-year-old ADPKD female received kidney transplantation 12 years ago from a living unrelated donor, and was maintained on cyclosporine and prednisolone as the only immunosuppressive drugs. The patient had a family history of ADPKD; two of her brothers died from ADPKD in youth, and one brother and two sisters received kidney transplants. She had an unremarkable clinical course till three months ago, when she was referred to our transplant service with right renal pain. Her consistent flank pain was continuous without hematuria or urinary tract infection. Ultra­sound of the native kidneys revealed a large solid tumor (22×11cm 2 ) in the right kidney, which involved the mid and lower pole, [Figure - 1]. The patient underwent a bilateral nephrec­tomy of the native kidneys. [Figure - 2] shows the large macroscopic tumor, which involved the mid and lower pole of the right polycystic kidney. Microscopic evaluation of the tumor suggested non-hodgkin's lymphoma, [Figure - 3].

Consequently, the patient received radiotherapy on the right side. The function of the transplanted kidney after three months of follow-up is still normal.


   Discussion Top


The incidence of benign renal adenomas in patients with ADPKD approximates 1 out of 4-5 patients (20-25%). [1],[4] Furthermore, The incidence of tumors in patients on immuno­suppressive therapy is 5 to 6%, or approxi­mately 100 times greater than that in the general population of the same age range. ADPKD is the only cause of renal failure that has a statistically significant association with malignancy in renal transplant recipients.[4],[6]

Renal cell carcinomas that develop in ADPKD patients is often bilateral, multicentric with sarcomatoid histology. [1],[7] Accordingly, bilateral nephrectomy is indicated. Lymphoma is one of the most common malignancies in transplantation, such as NHL. The risks are increased in proportion to the total immuno­suppressive load administered and time elapsed since transplantation. [8] Another predisposing factor for malignant changes is preexisting genetic mutations.[7]

Therefore, we suggest a regular sonographic screening of the native kidneys twice a year in all renal transplant patients. Post transplant nephrectomy can be performed with no mortality and should be carried out whenever clinically indicated. According to a review of the literature, this is the first report of NHL in ADPKD after kidney transplantation. Considering that NHL is sensitive to radiotherapy, we suggest tumor field radiotherapy after nephrectomy.

 
   References Top

1.Badani KK, Hemal Ak, Menon M. Autosomal dominant polycystic kidney disease and pain- review of the disease from etiology, evaluation, past surgical treatment options to current practice. J Postgrad Med 2004;50(3):222-6.  Back to cited text no. 1    
2.Grantham JJ. polycystic kidney disease: hereditary and acquired. Adv Intern Med 1993;38:409-20.  Back to cited text no. 2  [PUBMED]  
3.Gabow PA. Autosomal dominant polycystic kidney disease. N Engl J Med 1993;329: 332-42.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Keith DS, Torres VE, King BF, Zinki H, Farrow GM. Renal cell carcinoma in autosomal dominant polycystic kidney disease. J AM Soc Nephrol 1994;4:1661-9.  Back to cited text no. 4    
5.Errasti P, Manrique J, Lavilla J, et al. Autosomal dominant polycystic kidney disease: high prevalence of graft loss for death related malignancies and cardiovascular risk factors. Transplant Proc 2003;35(5):1717-9.  Back to cited text no. 5    
6.Gardner BP, Evans DB. Primary hepatocellular carcinoma arising in a renal transplant recipient with polycystic disease. J Postgrad Med 1983;59(688):120-1.  Back to cited text no. 6    
7.Delong MJ, Schmitt D, scott KM, Ramakumar S,Lien YH. Multicentric papillary renal carcinoma in renal allograft. Am J Kidney Dis 2003 Aug;42(2):381-4.  Back to cited text no. 7    
8.Carpenter CB, Milford EL, Sayegh MH. Transplantation in the treatment of renal failure. In: Kasper DL, Braunwald E, Fauci AS, Hauster SL, Longo DL, Jameson JL, editors. Harrison's principles of internal medicine 16 th ed. McGraw-Hill: Medical Publishing Division; 2005.p.1673.  Back to cited text no. 8    

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Correspondence Address:
Mohammad Ali Amirzargar
Department of Kidney Transplant, Ekbatan Hospital, Hamadan Medical University
Iran
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PMID: 17679756

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]

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    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures
 

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