Chronic lymphocytic leukaemia (CLL) is characterised by a lymphocytosis of mature-appearing clonal CD5+, CD23+ B lymphocytes. CLL cells arise from the bone marrow and infiltrate lymphoid tissues such as lymph nodes and spleen. Presentation is usually through discovery of lymphocytosis or lymphadenopathy. Unusual presentations, especially paraneoplastic syndromes are rare. Here, we describe a rare case presenting with severe nephrotic syndrome associated with the presence of a monoclonal protein in serum. Workup for suspected plasma cell dyscrasia led instead to the diagnosis of bone marrow infiltration by atypical CLL without lymphocytosis. Renal biopsy showed a glomerulonephritis that turned out to be paraneoplastic as it went into remission after treatment for CLL. Our case shows an unusual presentation of CLL and prompts for increased awareness of lymphoproliferative disorders in the context of seemingly unrelated conditions that may be paraneoplastic in origin.

A rare case of atypical chronic lymphocytic leukaemia presenting as nephrotic syndrome / M. Soldarini, L. Farina, A. Genderini, N. Bolli. - In: BMJ CASE REPORT. - ISSN 1757-790X. - 2017(2017 Jul 14). [10.1136/bcr-2016-218850]

A rare case of atypical chronic lymphocytic leukaemia presenting as nephrotic syndrome

M. Soldarini
Primo
;
L. Farina
Secondo
;
N. Bolli
Ultimo
2017-07-14

Abstract

Chronic lymphocytic leukaemia (CLL) is characterised by a lymphocytosis of mature-appearing clonal CD5+, CD23+ B lymphocytes. CLL cells arise from the bone marrow and infiltrate lymphoid tissues such as lymph nodes and spleen. Presentation is usually through discovery of lymphocytosis or lymphadenopathy. Unusual presentations, especially paraneoplastic syndromes are rare. Here, we describe a rare case presenting with severe nephrotic syndrome associated with the presence of a monoclonal protein in serum. Workup for suspected plasma cell dyscrasia led instead to the diagnosis of bone marrow infiltration by atypical CLL without lymphocytosis. Renal biopsy showed a glomerulonephritis that turned out to be paraneoplastic as it went into remission after treatment for CLL. Our case shows an unusual presentation of CLL and prompts for increased awareness of lymphoproliferative disorders in the context of seemingly unrelated conditions that may be paraneoplastic in origin.
haematology (drugs and medicines); nephrotic syndrome; Aged; Antineoplastic Combined Chemotherapy Protocols; Diagnosis, Differential; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Male; Nephrotic Syndrome; Paraneoplastic Syndromes; Medicine (all)
Settore MED/15 - Malattie del Sangue
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/607924
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