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| Year : 2011 | Volume
: 22
| Issue : 1 | Page : 126-129 |
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| Collagenofibrotic glomerulopathy in association with hodgkin's lymphoma |
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Sachin S Soni1, Swarnalata Gowrishankar2, Amit P Nagarik3, Sriganesh R Barnela3, Michelle De Padua2, Gopal Kishan Adikey3, Anuradha Raman3
1 Department of Nephrology, Mediciti Hospitals, Aurangabad, Maharashtra; Department of Nephrology, Manik Hospital and Research Centre, Aurangabad, India
2 Department of Histopathology, Apollo Health City, Hyderabad, Andhra Pradesh, India
3 Department of Nephrology, Mediciti Hospitals, Aurangabad, Maharashtra, India
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| Date of Web Publication | 30-Dec-2010 |
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 Abstract | | |
Deposit glomerulopathies are characterized by fibrillary deposits of various sizes, mainly in the mesangial area. Collagenofibrotic glomerulopathy is a rare type of such fibrillary glomerulopathies characterized by deposits of 60-80 nm fibrils in the sub-endothelial and mesangial areas. It is also associated with increased levels of serum pro-collagen type III peptide (PIIINP). Although most of the initial reports have emanated from Japan, many other scientists around the globe have later reported this disease. Possibility of systemic disease affecting metabolism of type III collagen is postulated but so far no such association has been identified. We report a 26-yearold male patient who presented with insidious onset of febrile illness associated with lymphadenopathy and proteinuria. Lymph node biopsy revealed features of Hodgkin's lymphoma while percutaneous renal biopsy showed features of collagenofibrotic glomerulopathy.
How to cite this article:
Soni SS, Gowrishankar S, Nagarik AP, Barnela SR, Padua MD, Adikey GK, Raman A. Collagenofibrotic glomerulopathy in association with hodgkin's lymphoma. Saudi J Kidney Dis Transpl 2011;22:126-9 |
How to cite this URL:
Soni SS, Gowrishankar S, Nagarik AP, Barnela SR, Padua MD, Adikey GK, Raman A. Collagenofibrotic glomerulopathy in association with hodgkin's lymphoma. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2020 Dec 6];22:126-9. Available from: https://www.sjkdt.org/text.asp?2011/22/1/126/74385 |
| Introduction | |  |
Collagenofibrotic glomerulopathy is an uncommonly described deposit glomerulopathy characterized by deposition of type III collagen fibrils within the mesangial matrix and subendothelial space and increase in serum procollagen type III peptide (PIIINP) levels. This rare glomerulopathy was first discussed in 1979 by Masaaki Arakawa at the meeting of the Japanese Society of Nephrology. [1] Subsequently, Arakawa and Yamanaka published a collection of ten cases from Japan. [2] We report a case of collagenofibrotic glomerulopathy in association with Hodgkin's lymphoma.
| Case Report | | |
A 26-year-old male patient presented with history of intermittent fever of six weeks duration, abdominal distension, nausea, anorexia and easy fatigability since one month and swelling over feet since two weeks. Clinical examination revealed marked pallor, bilateral pedal edema and normal blood pressure (120/70 mm Hg in sitting position). He also had multiple small posterior cervical lymph nodes (LNs) and a large right axillary LN, moderate splenomegaly and ascitis.
Laboratory investigations revealed severe anemia (hematocrit 24.6 vol%), leukocytosis (13400/mm 3 ), normal platelet count (3.81 lakhs/ mm 3 ), erythrocyte sedimentation rate of 124 mm at first hour, severe hypoalbuminemia (1.8 g/dL) and normal serum creatinine (1.3 mg/dL). Urine examination revealed 3+ dipstick proteinuria and bland urine sediment and 24-hour proteinuria was 1.6 g. Computed tomography (CT) scan of the abdomen revealed generalized intra-abdominal lymphadenopathy, renomegaly and splenomegaly with infiltration. His collagen profile and serological tests for Hepatitis B, Hepatitis C, Ebstein Barr virus and HIV were negative.
He underwent excision biopsy of the right axillary LN and percutaneous renal biopsy. Histopathology and immunohistochemistry of LN [Figure 1] confirmed it to be a case of mixed cellularity variant of Hodgkin's lymphoma. | Figure 1: Lymph node biopsy (high power view) (a) showing classical Reid Sternberg cell occupying center of the image; (b) immunohistochemistry showing positivity for CD30.
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Light microscopy of renal tissue [Figure 2] showed mild basement membrane thickening with mesangial widening. There was no evidence of tubular atrophy or interstitial fibrosis. Immunofluorescence study was negative for IgG, IgM, IgD, C3, kappa and lambda light chains. Electron microscopy showed flattening of foot processes, mild thickening of basement membrane in some capillaries and fibrillary deposits of 60-80 nm in size in mesangial area forming a fascicle [Figure 2]. Serum PIIINP levels could not be estimated due to lack of testing facility. Thus, a diagnosis of collagenofibrotic glomerulopathy in association with Hodgkin's lymphoma was made. This is the first case of this association being reported in literature. | Figure 2: Renal biopsy: (a) Light microscopy (high power) showing mild thickening of the basement membrane with mesangial widening; (b) electron microscopic image (×80,000 magnification) showing deposit in the mesangium forming a fascicle.
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| Discussion | | |
Collagenofibrotic glomerulopathy is a disorder of unknown etiology characterized by accumulation of atypical type III collagen fibrils within the mesangial matrix and sub-endothelial space and increased serum PIIINP levels. [3] Type III collagen is a structural protein of the extracellular matrix. In the human kidney, it is present only in the interstitium and blood vessels and not in the glomerulus.
The disease is rare, with around 40 published cases in the English literature. [4] Most of the initial reports were from Japan. However, Dombros from Canada, [5] Imbasciati from Italy, [6] Bernasovska from Slovakia [7] and Reis from Brazil [8] have reported isolated cases of this disease. In 1993, Gubler et al, reported 10 French children of this disorder, who had evidence for familial renal disease with an autosomal recessive transmission. [9]
The etiopathogenesis of this glomerulopathy is not well established. Clustering of the cases from Japan points to environmental or racial factors. Occurrence of the disease in siblings points to genetic etiology. [9],[1] Increased circulating levels of PIIINP and an autopsy report of collagen III deposition in all organs in a known case of collagenofibrotic glomerulopathy [11] suggest the possibility of systemic disorder with abnormal metabolism of type III collagen.
However, no disorder has been identified so far. Collageno-fibrotic glomerulopathy is being reported in association with a systemic disease (Hodgkin's lymphoma) for the first time. Many other glomerular diseases are reported in association with Hodgkin's lymphoma, namely, minimal change, membranous, membranoproliferative and crescentric glomerulonephritis as well as amyloidosis. [12] Mixed cellularity Hodgkin's disease is the histologic variety most frequently associated with the nephrotic syndrome, [13] as in our case.
Histologically, collagenofibrotic glomerulopathy presents as deposit glomerulopathy with eosinophilic sub-endothelial and mesangial deposits. The deposits are periodic acid-Schiff (PAS) and Congo red stain negative but positive for aniline blue and acid fuschin orange. [14] Immunofluorescence study for immunoglobulins, complement components and light chains is negative and characteristically positive for type III collagen. Positive immunostaining for type V collagen is also reported. [15] Electron microscopy is imperative to establish a definitive diagnosis which shows marked accumulation of fibrillar material in the mesangium and sub-endothelial space of size around 60 nm, as in our case. The fibers form irregularly arranged bundles and sometimes impart flower like appearance on cross section. [4]
The clinical presentation includes edema and/ or persistent proteinuria (nephrotic range in up to 60%), hypertension (in two-third of the cases) and anemia. Renal function test results usually are normal or slightly increased at presentation. [4] The case described also presented with edema and proteinuria leading to severe hypoalbuminemia and normal renal function.
No specific therapy is available. Steroid therapy is reported to be effective in temporarily treating the renal dysfunction, anemia and proteinuria. [16] The natural history is variable, but the disease is progressive, with progression to end-stage renal disease in 5-12 years. [17] Successful kidney transplantation has been reported with no recurrence at three years. [17] It will be interesting to see whether successful treatment of the systemic disease (Hodgkin's lymphoma) leads to resolution of renal disease in this case.
| Acknowledgments | | |
Dr. Arun Chitale from Jaslok Hospital, Mumbai, India, for electron microscopic and Dr. Sonali Saboo and Dr. M.Ch. Balaji Reddy from Focus Diagnostic Centre, Hyderabad, India, for radiologic assistance are acknowledged.
| References | | |
| 1. | Arakawa M, Hueki H, Sato M, Yamashita K, Nakashima S. Idiopathic mesangiodegenerative glomerulonephropathy: A proposal of a new glomerular disease. Jpn J Nephrol 1979;21:1404. 
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| 2. | Ikeda K, Yokoyama H, Tomosugi N, Kida H, Ooshima A, Kobayashi K. Primary glomerular fibrosis: A new nephropathy caused by diffuse intra-glomerular increase in atypical type III collagen fibers. Clin Nephrol 1990;33(4):155-9.
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| 3. | Abt AB, Cohen AH. Newer glomerular diseases. Semin Nephrol 1996;16:501-10.
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| 4. | Alchi B, Nishi S, Narita I, Gejyo F. Collagenofibrotic glomerulopathy: clinicopathologic overview of a rare glomerular disease. Am J Kidney Dis 2007;49(4):499-506.
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| 5. | Dombros N, Katz A. Nail-patella like renal lesion in the absence of skeletal abnormalities. Am J Kidney Dis 1982;1(4):237-40.
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| 6. | Imbasciati E, Gheradi G, Morozumi K, et al. Collagen Type III glomerulopathy: A new idiopathic glomerular disease. Am J Nephrol 1991;11(5):422-9.
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| 7. | Bernasovska G, Demes M, Oksa A, et al. Collagenofibrotic glomerulopathy-rare glomerulonephritis. Vnitr Lek 2006;52(12):1200-4.
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| 8. | Bichuette-Custodio F1, Castro E1, Teixeira V, Reis MA. Collagenofibrotic glomerulopathy: A description of two cases. Abstract book ofWorld Congress of Nephrology. 2007. p. 327.
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| 9. | Gubler MC, Dommergues JP, Foulard M, et al. Collagen type III glomerulopathy: A new type of hereditary nephropathy. Pediatr Nephrol. 1993;7(4):354-60.
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| 10. | Tamura H, Matsuda A, Kidoguchi N, Matsumura O, Mitarai T, Isoda K. A family with two sisters with collagenofibrotic glomerulonephropathy. Am J Kidney Dis 1996;27(4):588-595.
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| 11. | Yasuda T, Imai H, Nakamoto Y, et al. Collagenofibrotic glomerulopathy: A systemic disease. Am J Kidney Dis 1999;33(1):123-7.
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| 12. | Davison AM, Hartley B. Malignancy associated glomerular disease. In: Davison AM, Cameronn JS, Grunfeld JP, et al, (eds). Textbook of Clinical Nephrology. 3rd ed. Oxford. Oxford University Press, 2005,634.
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| 13. | Moorthy AV, Zimmennan SW, Burkholder PM. Nephrotic syndrome in Hodgkin's disease: Evidence for pathogenesis alternative to immune complex deposition. Am J Med 1976;61(4): 471-7.
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| 14. | Kern WF, Silva FG, Laszik ZG, Bane BL, Nadasdy T, Pitha JV. Miscellaneous conditions affecting the kidney. In: Kern WF, Silva FG, Laszik ZG, Bane BL, Nadasdy T, Pitha JV, (eds). Atlas of Renal Pathology. 1 st ed. Philadelphia, PA. Saunders, 1999, 194-207.
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| 15. | Morita H, Hasegawa T, Minamoto T. Collagenofibrotic glomerulopathy with a widespread expression of type-V collagen. Virchows Arch 2003;442(2):163-8.
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| 16. | Hisakawa N, Yasuoka N, Nishiya K. Collagenofibrotic glomerulonephropathy associated with immune complex deposits. Am J Nephrol 1998;18(2):134-41.
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| 17. | Suzuki T, Okubo Y, Ikezumi Y. A favorable course of collagenofibrotic glomerulopathy after kidney transplantation and questionnaire survey about the prognosis of collagenofibrotic glomerulopathy. Jpn J Nephrol 2004;46(4): 360-4.
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Correspondence Address:
Anuradha Raman
Director of Nephrology, Mediciti Hospitals, 5-9-22, Secretariat Road, Hyderabad 500063, Andhra Pradesh
India
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PMID: 21196628 
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