Simultaneous clinical resolution of focal segmental glomerulosclerosis associated with chronic lymphocytic leukaemia treated with fludarabine, cyclophosphamide and rituximab

BMC Nephrology

Ioannis Stefanidis³  Panagiota Matsouka¹  Foteini Karasavvidou²  Panagiota Kourti³  Theodoros Eleftheriadis³  Vassilios Liakopoulos³  Nikolaos Giannakoulas¹  Spyridon Arampatzis³ 

[1]Department of Haematology, University of Thessaly, Larissa, Greece

[2]Department of Pathology, University of Thessaly, Larissa, Greece

[3]Department of Nephrology, University of Thessaly, Larissa, Greece

关键词: rituximab;    cyclophosphamide;    fludarabine;    nephrotic syndrome;    focal segmental glomerulosclerosis;    chronic lymphocytic leukemia;   

Others  :  1083250
DOI  :  10.1186/1471-2369-12-33

 received in 2011-03-30, accepted in 2011-07-05,  发布年份 2011

【 摘 要 】

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.

【 授权许可】

   
2011 Arampatzis et al; licensee BioMed Central Ltd.

【 图 表 】

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