Central Nervous System Expression of a Monoclonal Paraprotein in a Chronic Lymphocytic Leukemia Patient

Ruiz, Phillip; Moezzi, Manijeh; Chamizo, Wilfredo; Ganjei, Parvin; Whitcomb, Clarence C.; Rey, Luís

doi:10.1159/000204593

Cited by 12 publications

(12 citation statements)

References 3 publications

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“…Four additional patients had hematopoietic malignancies (lymphomas in 3 and chronic lymphocytic leukemia in one patient). Although reported in one case [15], single CSF bands in such systemic disorders seem to be incidental rather than disease-related.…”

Section: Discussionmentioning

confidence: 71%

A multicenter study on the diagnostic significance of a single cerebrospinal fluid IgG band

et al. 2017

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The analysis of paired cerebrospinal fluid (CSF) and serum samples with isolectric focusing (IEF) can yield different patterns which can be of aid in the differential diagnosis of central nervous system (CNS) disorders. Rarely, a single CSF-restricted IgG band, which is not included within these patterns, can be detected in association with inflammatory disorders, multiple sclerosis (MS) above all. However, the diagnostic meaning of this abnormality is still uncertain. The main aim of our multicenter study was to establish the frequency and disease associations of single CSF IgG bands. Differences in the CSF profiles between MS and other diseases, and the follow-up patterns were also evaluated. Medical records of patients who underwent CSF analysis, which included IEF, over a 11.5-year period were retrospectively scrutinized at the participating centers, which used similar IEF techniques. One hundred and fifty-one of 9422 CSF reports (1.6%) showed single CSF-restricted IgG bands. Of the 129 patients with a definite diagnosis, 58.2% had CNS inflammatory-demyelinating diseases (the most frequent being MS: 21.7%), 6.2% tumours, 5.4% inflammatory peripheral nervous system diseases and 30.2% miscellaneous diseases. At follow-up, 3 out of the 10 patients with a repeated CSF analysis had developed an oligoclonal band pattern. Our findings indicate that single CSF IgG bands tend to associate with diseases characterized by the involvement of intrathecal humoral immune responses, and strongly support the notion that this abnormality should be regularly reported, thus alerting clinicians of possible inflammatory disorders of the CNS.

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Section: Discussionmentioning

confidence: 71%

A multicenter study on the diagnostic significance of a single cerebrospinal fluid IgG band

et al. 2017

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“…[3][4][5][6][7][8][9][10][11][12][13][14][15][16] Among the 4174 CLL patients cared for at our center over a 17-year interval, 33 (19% of evaluated patients; 0.8% overall cohort) developed clinically significant CNS involvement by CLL or RS. While clinically significant CNS involvement by CLL is rare, neurological symptoms frequently occur in patients with CLL, and distinguishing whether or not these symptoms are due to CLL or other etiologies can be challenging.…”

Section: Discussionmentioning

confidence: 99%

“…[3][4][5][6][7][8][9][10][11][12][13][14][15][16] Despite the low frequency of clinically significant CNS involvement by CLL, post-mortem studies of patients with CLL indicate that occult CNS involvement by CLL is a relatively common finding, with a prevalence of 7%-71%. [17][18][19][20] This discrepancy between clinical manifestations and autopsy findings illustrates the fact that, while CLL cells may frequently be present in the CNS, they rarely cause clinically significant manifestations.…”

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confidence: 99%

Prevalence and characteristics of central nervous system involvement by chronic lymphocytic leukemia

et al. 2016

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A broad array of conditions can lead to neurological symptoms in chronic lymphocytic leukemia patients and distinguishing between clinically significant involvement of the central nervous system by chronic lymphocytic leukemia and symptoms due to other etiologies can be challenging. Between January 1999 and November 2014, 172 (4%) of the 4174 patients with chronic lymphocytic leukemia followed at our center had a magnetic resonance imaging of the central nervous system and/or a lumbar puncture to evaluate neurological symptoms. After comprehensive evaluation, the etiology of neurological symptoms was: central nervous system chronic lymphocytic leukemia in 18 patients (10% evaluated by imaging and/or lumbar puncture, 0.4% overall cohort); central nervous system Richter Syndrome in 15 (9% evaluated, 0.3% overall); infection in 40 (23% evaluated, 1% overall); autoimmune/inflammatory conditions in 28 (16% evaluated, 0.7% overall); other cancer in 8 (5% evaluated, 0.2% overall); and another etiology in 63 (37% evaluated, 1.5% overall). Although the sensitivity of cerebrospinal fluid analysis to detect central nervous system disease was 89%, the specificity was only 42% due to the frequent presence of leukemic cells in the cerebrospinal fluid in other conditions. No parameter on cerebrospinal fluid analysis (e.g. total nucleated cells, total lymphocyte count, chronic lymphocytic leukemia cell percentage) were able to offer a reliable discrimination between patients whose neurological symptoms were due to clinically significant central nervous system involvement by chronic lymphocytic leukemia and another etiology. Median overall survival among patients with clinically significant central nervous system chronic lymphocytic leukemia and Richter syndrome was 12 and 11 months, respectively. In conclusion, clinically significant central nervous system involvement by chronic lymphocytic leukemia is a rare condition, and neurological symptoms in patients with chronic lymphocytic leukemia are due to other etiologies in approximately 80% of cases. Analysis of the cerebrospinal fluid has high sensitivity but limited specificity to distinguish clinically significant chronic lymphocytic leukemia involvement from other etiologies.

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“…For instance, involvement of the central nervous system (CNS) in B-CLL is very rare; so far only 14 cases have been reported in the literature. [2][3][4][5][6][7][8][9][10][11][12][13][14] Chlorambucil has been the first-choice agent for the treatment of B-CLL. Other chemotherapeutic agents, including cyclophosphamide, 6-mercaptopurine (6-MP), vincristine, or combination therapy with vincristine, cyclophosphamide, prednisolone, and adriamycin (VEPA) have also been used.…”

Section: Introductionmentioning

confidence: 99%

Excellent response of chemotherapy-resistant B-cell-type chronic lymphocytic leukemia with meningeal involvement to rituximab

et al. 2005

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A 70-year-old woman was diagnosed with B-cell-type chronic lymphocytic leukemia (B-CLL) in May 2001. Initial white blood cell (WBC) count was 37 x 10(9)/l and most of the cells were mature small lymphocytes. Surface antigen analysis of these lymphocytes revealed positive reactions for CD19, 20, 25, 5, and lambda-light chain. Despite her Rai stage-0 status, various treatments were ineffective, including cyclophosphamide; fludarabine; 6-mercaptopurine; a combination of vincristine, cyclophosphamide, prednisolone, and adriamycin; and etoposide. Her WBC count increased, ranging from 150 to 450 x 10(9)/l, with marked splenomegaly, and symptoms of meningitis, such as headache, ophthalmalgia, hearing disturbance, and abnormal behavior, being manifested. The WBC count in the cerebrospinal fluid was elevated to 134/microl. The surface phenotype of these cells was identical to that of circulating lymphocytes, indicating meningeal involvement of leukemia, a rare complication in B-CLL. At the time of this WBC elevation, 24% of circulating lymphocytes had prominent nucleoli, indicating progression of the disease to CLL/prolymphocytic leukemia. Her symptoms disappeared after repeated intrathecal injections of methotrexate and dexamethazone. After four courses of treatment of the refractory B-CLL with rituximab, an anti-CD20 monoclonal antibody, the WBC count returned to normal levels and the splenomegaly disappeared. She is currently well, with sustained remission, as of April 2004.

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Central Nervous System Expression of a Monoclonal Paraprotein in a Chronic Lymphocytic Leukemia Patient

Cited by 12 publications

References 3 publications

A multicenter study on the diagnostic significance of a single cerebrospinal fluid IgG band

A multicenter study on the diagnostic significance of a single cerebrospinal fluid IgG band

Prevalence and characteristics of central nervous system involvement by chronic lymphocytic leukemia

Excellent response of chemotherapy-resistant B-cell-type chronic lymphocytic leukemia with meningeal involvement to rituximab

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