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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 4  |  Page : 724-727
Renal lymphangiectasia associated with chronic myeloid leukemia

Yash Diagnostic Center, Yash Hospital and Research Center, Civil Lines, Kanth Road, Moradabad (UP), India

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Date of Web Publication26-Jun-2010


Renal lymphangiectasia is a rare disorder characterized by dilatation of peripelvic, renal and perirenal lymphatic ducts. The exact etiology is not known. Congenital forms and ac­quired forms have been described. The latter has been attributed to obstruction of draining retro­peritoneal lymphatic ducts caused by either infection, inflammation or any other cause. We des­cribe the rare association of renal lymphangiectasia with chronic myeloid leukemia, which is probably not yet reported in the medical literature.

How to cite this article:
Rastogi R, Rastogi U C, Sarikwal A, Rastogi V. Renal lymphangiectasia associated with chronic myeloid leukemia. Saudi J Kidney Dis Transpl 2010;21:724-7

How to cite this URL:
Rastogi R, Rastogi U C, Sarikwal A, Rastogi V. Renal lymphangiectasia associated with chronic myeloid leukemia. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2020 Oct 30];21:724-7. Available from: https://www.sjkdt.org/text.asp?2010/21/4/724/64660

   Introduction Top

Renal lymphangiectasia is a rare, benign mal­formation That results from a lack of commu­nication between the lymphatic ducts draining the renal and perirenal area with the retrope­ritoneal lymphatics. [1],[2] Subsequent dilatation of lymphatic ducts results in formation of unilo­cular or multilocular perineprhic fluid collec­tion. Few reports have been described in the medical literature. [2],[3]

   Case Report Top

A 60-year-old man who lives in a village pre­sented with a complaint of pain and tightness of abdomen was referred for evaluation at our center. Past medical history of high-grade fever was negative. Previous medical records of one year back including ultrasonography of the ab­domen were unremarkable. Clinical examina­tion revealed cachexia and hepatosplenomegaly. Recent laboratory tests revealed raised total leukocyte count (TLC) greater than 50,000 cells/ mm 3 , raised ESR to 65 mm, and normal renal function tests.

Ultrasonography of the abdomen revealed mild hepatomegaly and massive splenomegaly. The inferior pole of the spleen was reaching below the level of umbilicus. In addition, both kidneys were enlarged with increased paren­chymal echogenicity and decreased corticome­dullary differentiation. There was an evidence of non-septate, perinephric anechoic collection around both kidneys; more collection was noticed on the right side [Figure 1]. The rest of the abdomen was unremarkable. Based on the ultrasonographic findings, the possibility of mye­loid leukemia with bilateral renal lymphangiec­tasia was considered. The patient was advised to obtain a complete peripheral blood exami­nation especially for immature cells and a con­trast enhanced computed tomography scan of the abdomen.

The complete examination of peripheral blood revealed TLC of 65, 000 cells/mm 3 with pre­sence of immature cells including myelocytes and metamyelocytes (greater than 10%) with a shift to left side. The pathologic diagnosis was compatible with chronic myeloid leukemia.

The computed tomography of the abdomen revealed bilateral nephromegaly with fluid atte­nuating perinephric collection around both kid­neys; with more collection of fluid on the right side [Figure 2], [Figure 3]a and b. An evidence of fluid filled structures was also shown in the renal pelvis. In addition, hepatomegaly and spleno­megaly was also noted with craniocaudal span of 18.2 cm and 21.4 cm, respectively. No evi­dence of any focal lesion was noted in the kidneys, liver, or spleen. No evidence of free fluid was noted in the peritoneal or pleural cavities. The rest of the abdomen was unre­markable.

Based on the clinical, pathological, and radio­logical findings, the diagnosis of chronic mye­loid leukemia with renal lymphangiectasia due to obstruction of the retroperitoneal lymphatic ducts by leukemic cells was suggested.

The patient underwent aspiration of the pe­rinephric fluid for cytologic and biochemical examination in addition to renal biopsy to rule out renal leukemic infiltration. The ultrasound­guided aspiration of the perinephric fluid from both sides was straw colored and revealed few cells per cubic mm and all were lymphocytes with no evidence of any leukemic cells. In addi­tion, the fluid was rich in proteins and renin and was negative for pus or bacteria. The renal biopsy for leukemic infiltration was negative.

   Discussion Top

Renal lymphangiectasia is a rare benign lym­phatic malformation. [3] The condition arises due to failure of renal and perirenal lymph to drain in to the retroperitoneal lymphatic ducts. The condition can be seen in children as well as adults with no age predilection. [4]

The condition may be asymptomatic as seen in our case or may present with nonspecific abdominal or flank pain, ascites, hypertension, and sometimes hematuria. [2],[5] The renal function is usually preserved.

The exact etiology of renal lymphangiectasia is unknown. Familial cases have been reported in the literature supporting the congenital nature of this disease. [6] However, the majority of the cases reported in literature including ours do not show any familial association, justifying the hypothesis of an acquired etiology. Obstruc­tion of lymphatic ducts secondary to infection, inflammation or any other cause produces dila­tation of peripelvic, intrarenal, and perirenal lymphatics. [7]

Renal lymphangiectasia may be associated with renal vein thrombosis, hypertension and its exacerbation has been reported during preg­nancy. [2],[4],[6] In our case, we hypothesize that the leukemic cells must have clogged the lympha­tic ducts in the retroperitoneum with resultant stasis of lymph from renal and perirenal region subsequently resulting in lymphangiectasia. To our knowledge, this is probably the first case of association of renal lymphangiectasia with chronic myeloid leukemia.

On excretory urogram in classical cases, re­nal lymphangiectasia presents as bilateral neph­romegaly (due to dilatation of the intra-renal lymphatics) with distension of the pelvicali­ceal system (due to dilated peripelvic lympha­tics) and distortion of the renal contour (due to perirenal collection). On cross-sectional ima­ging, renal lymphangiectasia appears as fluid collections in the peripelvic and perirenal areas and nephromegaly with or without altered cor­ticomedullary differentiation. [8] The presence of fluid filled structures in the retroperitoneum may also be detected. [4],[6],[9] Unilateral involvement is occasionally seen, [10] as well as the presentation of renal lymphangioma as a solid focal lesion on US and CT. [11] There may be associated dis­placement of the adjacent structures, but no evidence of invasion is seen differentiating it from malignant processes. Though definitive diagnosis can be made only by microscopic and biochemical analysis of the aspirated lymph, [4] imaging allows accurate diagnosis in majo­rity of the cases.

The important differential diagnosis on cross­sectional imaging with lymphangiectasia in­cludes multiple cortical cysts as seen in adult polycystic kidney disease, von Hippel-Lindau disease, or multilocular cystic nephroma. In both conditions, cystic lesions may additio­nally be seen in liver and pancreas and careful examination will show multiple cysts scattered nonuniformly in the renal parenchyma rather a unilocular or multilocular perirenal collection. Furthermore, focal deposits in lymphoma, leu­kemia and nephroblastomatosis may mimic lymphangiectasia, but the focal lesions in these conditions have soft tissue appearance rather than fluid nature on imaging. In addition, the presence of lymph nodes in the retroperito­neum favors the diagnosis of lymphoma. Uri­noma and perinephric abscesses also should be ruled out. [5]

Though the natural course of this disease is unknown, treatment is usually not necessary. Complicated cases with altered renal function, hypertension, etc may be an indication for gui­ded aspiration, marsupialisation and rarely nephrectomy. [4],[5] One case of partial regression was reported in a neonate. [12]

We conclude that renal lymphangiectasia is a rare lymphatic dilatation that can be congenital or acquired. Chronic myeloid leukemia can be one of the acquired causes producing renal lymphangiectasia probably by obstructing the drainage of the renal and perirenal region in to the larger retroperitoneal lymphatic ducts.

   Acknowledgement Top

We are highly thankful to Mr. Shivam and Ms. Anju for their kind help in acquisition of images.

   References Top

1.Leder RA, Frederik MG, Hall BP, Elenberger CD. Genito-urinary case of the day: Renal lymphangiomatosis. AJR Am J Roentgenol 1995;165:197-200.  Back to cited text no. 1      
2.Rajeev TP, Barua S, Deka PM, Hazarika S. Bilateral perirenal lymphangiomatosis. Indian J Urol 2006;22:73-4.  Back to cited text no. 2    Medknow Journal  
3.Yasutomo N, Yukiomi N, Hideki S. Renal lymphangioma. J Urol 1999;162:484-5. 4. Riehl J, Schmitt H, Schafer L, Schneider B,  Back to cited text no. 3      
4.Sieberth HG. Retroperitoneal lymphangiectasia associated with bilateral renal vein thrombosis. Nephrol Dial Transplant 1997;12:1701-3.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Ashraf K, Raza SS, Ashraf O, Memon W, Memon A, Zubairi TA. Renal lymphangiec­tasia. Br J Radiol 2007;80:e117-8.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Meredith WT, Levine E, Ahlstom NG, Gran­tham JJ. Exacerbation of familial renal lym­phangiomatosis during pregnancy. AJR Am J Roentgenol 1988;151:965-6.  Back to cited text no. 6      
7.Kutcher R, Mahadevia P, Nussbaum MK, Rosenblatt R, Freed S. Renal peripelvic multic­ystic lymphangiectasia. Urology 1987;30:177-9.  Back to cited text no. 7  [PUBMED]    
8.Mani NB, Sodhi KS, Singh P, Katariya S, Poddar U, Thapa BR. Renal lymphangioma­tosis: A rare cause of bilateral nephromegaly. Aust Radiol 2003;47:184-7.  Back to cited text no. 8      
9.Varela JR, Bargiela A, Requejo I, Fernandez R, Darriba M, Pombo F. Bilateral renal lym­phangiomatosis: US and CT findings. Eur Radiol 1998;8:230-1.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Gupta R, Sharma R, Gamanagatti S, Dogra PN, Kumar A. Unilateral renal lymphangiectasia: Imaging appearance on sonography, CT and MRI. Int Urol Nephrol 2007;39:361-4.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]  
11.Kim JK, Ahn JH, Kim KR, Cho KS. Renal lymphangioma manifested as a solid mass on ultrasonography and computed tomography. J Ultrasound Med 2002;21:203-6.  Back to cited text no. 11      
12.Pickering SP, Fletcher BD, Bryan PJ, Abra­mowsky CR. Renal lymphangioma: a cause of neonatal nephromegaly. Pediatr Radiol 1984; 14: 445-8.  Back to cited text no. 12      

Correspondence Address:
Rajul Rastogi
Yash Diagnostic Center, Yash Hospital and Research Center, Civil Lines, Kanth Road, Moradabad (UP)
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