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Simultaneous clinical resolution of focal segmental glomerulosclerosis associated with chronic lymphocytic leukaemia treated with fludarabine, cyclophosphamide and rituximab.
BMC Nephrol. 2011 Jul 05; 12:33.BN

Abstract

BACKGROUND

Although renal involvement in advanced haematological malignancies is common, glomerulonephritis associated with lymphoproliferative disorders is rare, and the related pathogenetic mechanisms are still poorly understood. We present a rare case of chronic lymphocytic leukaemia(CLL)-associated focal segmental glomerulosclerosis with nephrotic-range proteinuria.

CASE PRESENTATION

A 53-year-old Caucasian man, previously healthy, with no history of hypertension, alcohol use or smoking presented with rapid weight gain, massive peripheral oedema, and hypertension. Laboratory findings included a white blood cell count of 49,800 cells/mm3 with an absolute lymphocyte count of 47,000 cells/mm3, serum albumin of 2.3 g/dL, urea 65 mg/dL, and creatinine 1.5 mg/dL. A 24-hour urine collection contained 7.1 g protein and significant haematuria. A peripheral blood smear showed mature lymphocytosis and smudge cells. Diagnostic imaging showed mild paraaortic lymphadenopathy with no renal abnormalities. Bone marrow aspiration and trephine biopsy showed diffuse and focal infiltration with B-CLL lymphocytes. Percutaneous renal biopsy revealed total sclerosis in 3/21(14%) of the glomeruli and focal and segmental solidification and sclerosis in 4/21 (19%) glomeruli. A regimen of fludarabine, cyclophosphamide and rituximab was successful in inducing remission of the CLL and clinical resolution of the nephritic-range proteinuria.

CONCLUSIONS

A multidisciplinary approach to monitor both the malignancy and the glomerular lesions is crucial for the optimal management of paraneoplastic glomerulonephritis. Although chemotherapy with fludarabine, cyclophosphamide and rituximab successfully treated CLL-associated nephrotic syndrome in our patient, further studies are required to confirm efficacy in this setting.

Authors+Show Affiliations

Department of Nephrology, University of Thessaly, Larissa, Greece. spiros.arampatzis@gmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

21729272

Citation

Arampatzis, Spyridon, et al. "Simultaneous Clinical Resolution of Focal Segmental Glomerulosclerosis Associated With Chronic Lymphocytic Leukaemia Treated With Fludarabine, Cyclophosphamide and Rituximab." BMC Nephrology, vol. 12, 2011, p. 33.
Arampatzis S, Giannakoulas N, Liakopoulos V, et al. Simultaneous clinical resolution of focal segmental glomerulosclerosis associated with chronic lymphocytic leukaemia treated with fludarabine, cyclophosphamide and rituximab. BMC Nephrol. 2011;12:33.
Arampatzis, S., Giannakoulas, N., Liakopoulos, V., Eleftheriadis, T., Kourti, P., Karasavvidou, F., Matsouka, P., & Stefanidis, I. (2011). Simultaneous clinical resolution of focal segmental glomerulosclerosis associated with chronic lymphocytic leukaemia treated with fludarabine, cyclophosphamide and rituximab. BMC Nephrology, 12, 33. https://doi.org/10.1186/1471-2369-12-33
Arampatzis S, et al. Simultaneous Clinical Resolution of Focal Segmental Glomerulosclerosis Associated With Chronic Lymphocytic Leukaemia Treated With Fludarabine, Cyclophosphamide and Rituximab. BMC Nephrol. 2011 Jul 5;12:33. PubMed PMID: 21729272.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Simultaneous clinical resolution of focal segmental glomerulosclerosis associated with chronic lymphocytic leukaemia treated with fludarabine, cyclophosphamide and rituximab. AU - Arampatzis,Spyridon, AU - Giannakoulas,Nikolaos, AU - Liakopoulos,Vassilios, AU - Eleftheriadis,Theodoros, AU - Kourti,Panagiota, AU - Karasavvidou,Foteini, AU - Matsouka,Panagiota, AU - Stefanidis,Ioannis, Y1 - 2011/07/05/ PY - 2011/03/30/received PY - 2011/07/05/accepted PY - 2011/7/7/entrez PY - 2011/7/7/pubmed PY - 2012/5/26/medline SP - 33 EP - 33 JF - BMC nephrology JO - BMC Nephrol VL - 12 N2 - BACKGROUND: Although renal involvement in advanced haematological malignancies is common, glomerulonephritis associated with lymphoproliferative disorders is rare, and the related pathogenetic mechanisms are still poorly understood. We present a rare case of chronic lymphocytic leukaemia(CLL)-associated focal segmental glomerulosclerosis with nephrotic-range proteinuria. CASE PRESENTATION: A 53-year-old Caucasian man, previously healthy, with no history of hypertension, alcohol use or smoking presented with rapid weight gain, massive peripheral oedema, and hypertension. Laboratory findings included a white blood cell count of 49,800 cells/mm3 with an absolute lymphocyte count of 47,000 cells/mm3, serum albumin of 2.3 g/dL, urea 65 mg/dL, and creatinine 1.5 mg/dL. A 24-hour urine collection contained 7.1 g protein and significant haematuria. A peripheral blood smear showed mature lymphocytosis and smudge cells. Diagnostic imaging showed mild paraaortic lymphadenopathy with no renal abnormalities. Bone marrow aspiration and trephine biopsy showed diffuse and focal infiltration with B-CLL lymphocytes. Percutaneous renal biopsy revealed total sclerosis in 3/21(14%) of the glomeruli and focal and segmental solidification and sclerosis in 4/21 (19%) glomeruli. A regimen of fludarabine, cyclophosphamide and rituximab was successful in inducing remission of the CLL and clinical resolution of the nephritic-range proteinuria. CONCLUSIONS: A multidisciplinary approach to monitor both the malignancy and the glomerular lesions is crucial for the optimal management of paraneoplastic glomerulonephritis. Although chemotherapy with fludarabine, cyclophosphamide and rituximab successfully treated CLL-associated nephrotic syndrome in our patient, further studies are required to confirm efficacy in this setting. SN - 1471-2369 UR - https://www.unboundmedicine.com/medline/citation/21729272/Simultaneous_clinical_resolution_of_focal_segmental_glomerulosclerosis_associated_with_chronic_lymphocytic_leukaemia_treated_with_fludarabine_cyclophosphamide_and_rituximab_ L2 - https://www.biomedcentral.com/1471-2369/12/33 DB - PRIME DP - Unbound Medicine ER -