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LETTERS TO EDITOR  
Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 300-301
Antineutrophil cytoplasmic antibody-associated vasculitis and chronic lymphocytic leukemia: A rare association


Department of Nephrology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

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Date of Web Publication19-Jun-2017
 

How to cite this article:
Nagarajan M, Dhanapriya J, Dineshkumar T, Sakthirajan R, Balasubramaniyan T, Gopalakrishnan N. Antineutrophil cytoplasmic antibody-associated vasculitis and chronic lymphocytic leukemia: A rare association. Indian J Pathol Microbiol 2017;60:300-1

How to cite this URL:
Nagarajan M, Dhanapriya J, Dineshkumar T, Sakthirajan R, Balasubramaniyan T, Gopalakrishnan N. Antineutrophil cytoplasmic antibody-associated vasculitis and chronic lymphocytic leukemia: A rare association. Indian J Pathol Microbiol [serial online] 2017 [cited 2022 Jun 30];60:300-1. Available from: https://www.ijpmonline.org/text.asp?2017/60/2/300/208387


Editor,

Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is a chronic lymphoproliferative disorder and has the propensity to develop autoantibodies against any cell lineage of bone marrow, commonly present as autoimmune hemolytic anemia and thrombocytopenia.[1] Rarely, it can induce autoantibodies against neutrophils which may manifest as crescentic glomerulonephritis. We report here a female patient with CLL and vasculitis related to antineutrophil cytoplasmic antibodies (ANCAs) presenting with rapidly progressive glomerulonephritis.

A 40-year-old female presented with progressive cervical lymphadenopathy with fever and night sweats of 1-month duration. The evaluation revealed total leukocyte count of 59,000/cumm and smudge cells in peripheral smear. Immunophenotyping of bone marrow showed CD5, CD19, CD23 positive and CD10, CD20, CD79b, CD38, and Zap-70 negative B-cell clone in 85% of bone marrow leukocytes. She was diagnosed to have CLL-Rai stage 1. She was started on chemotherapy with intravenous (IV) cyclophosphamide with prednisolone 1 mg/kg/day along with IV bendamustine 100 mg IV for 2 days in a month. She was under remission for the next 1 year after which she developed oliguric renal failure. Laboratory investigations revealed urine analysis: 3+ proteinuria with 5–6 red blood cells/hpf; urine protein creatinine ratio: 4.5; blood hemoglobin: 6.2 g/dl; total leukocyte count: 23,200 cells/m 3; differential count: polymorph 38% and lymphocytes 62%; platelet count: 3.2 lakhs/cumm; blood urea: 119 mg/dl; serum creatinine: 6 mg/dl; serum uric acid: 8.4 mg/dl; corrected serum calcium: 7.5 mg/dl; serum phosphorus: 5.9 mg/dl; serum sodium: 135 meq/l; potassium: 4.1 meq/l; and serum lactate dehydrogenase: 242 U/l. Viral markers (hepatitis B surface antigen, anti-hepatitis C virus, and HIV) were negative. Antinuclear antibodies and dsDNA were negative. Serum complements C3 (97.5 mg/dl) and C4 (24.4 mg/dl) were normal. c-ANCA (anti-PR-3) was positive by indirect IF and ELISA. Ultrasound examination revealed renomegaly. Renal biopsy showed segmental necrosis [Figure 1] of capillary tuft in 5 out of 13 glomeruli, with cellular crescents in 6 glomeruli. Dense inflammatory infiltrates throughout the core and sheets of lymphocytes in renal capsular and perirenal soft tissue. Immunofluorescence was negative for C3, IgG, IgM, IgA, and C1q. Pauci-immune (ANCA-associated) crescentic glomerulonephritis along with lymphocytic infiltration of interstitium due to CLL/SLL flare was diagnosed. She was treated with pulse IV methyl prednisolone (1000 mg/day) for 3 days followed by oral prednisolone of 1 mg/kg/day which tapered after 1 month and IV cyclophosphamide 500 mg/m 2 pulse fortnightly. She had persistent dialysis dependent renal failure even at the end of 3 months. Six months later, she died of fulminant bacterial sepsis.
Figure 1: Renal biopsy showing segmental necrosis of glomerulus (silver stain)

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The renal manifestation of CLL is varied. Only <2% of CLL patients have detectable ANCA titers.[2] Nephrotic syndrome occurs in 1%–2% and is considered to be a true paraneoplastic syndrome secondary to membranoproliferative glomerulonephritis (33.7%) and membranous nephropathy (19%).[3] Cryoglobulin secreting B-cell clone is identified in 50% of CLL with renal involvement. Minimal change, focal segmental sclerosis, focal glomerulonephritis, amyloidosis, immunotactoid, and crescentic glomerulonephritis have also been described.

Our patient presented with pauci-immune ANCA (c-ANCA)-associated crescentic glomerulonephritis with CLL/SLL. Till date, only a few such case reports have been published. Among five cases reported in literature [Table 1], four had p-ANCA positive and one had c-ANCA positive. Crescentic glomerulonephritis was seen in four patients. The time duration between the occurrence of ANCA-mediated crescentic glomerulonephritis and CLL is varied. The aim of management is usually to achieve remission of CLL. Most of the reported cases of ANCA vasculitis in CLL had poor outcome.[4],[5] Overall patient survival was influenced by CLL and its complications rather than renal disease.
Table 1: Antineutrophil cytoplasmic antibody positisvity and renal biopsy in chronic lymphocytic leukemia patients in the literature

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Acknowledgment

We would like to thank Dr. Anila Abraham Kurien MD Renopath, Chennai for her help in evaluating renal biopsy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Ghia P, Scielzo C, Frenquelli M, Muzio M, Caligaris-Cappio F. From normal to clonal B cells: Chronic lymphocytic leukemia (CLL) at the crossroad between neoplasia and autoimmunity. Autoimmun Rev 2007;7:127-31.  Back to cited text no. 1
[PUBMED]    
2.
Cil T, Altintas A, Isikdogan A, Batun S. Prevalence of antineutrophil cytoplasmic antibody positivity in patients with Hodgkin's and non-Hodgkin lymphoma: A single center experience. Int J Hematol 2009;90:52-7.  Back to cited text no. 2
[PUBMED]    
3.
Cambier JF, Ronco P. Onco-nephrology: Glomerular diseases with cancer. Clin J Am Soc Nephrol 2012;7:1701-12.  Back to cited text no. 3
    
4.
Rivera M, González C, Gonzalo A, Quereda C, Fogué L, Ortuño J. Vasculitis associated with non-Hodgkin's lymphoma. Nephron 1993;65:167-8.  Back to cited text no. 4
    
5.
Dussol B, Brunet P, Vacher-Coponat H, Bouabdallah R, Chetaille P, Berland Y. Crescentic glomerulonephritis with antineutrophil cytoplasmic antibodies associated with chronic lymphocytic leukaemia. Nephrol Dial Transplant 1997;12:785-6.  Back to cited text no. 5
    

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Correspondence Address:
Jeyachandran Dhanapriya
Department of Nephrology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.208387

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