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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Haematologica
2015 ; 100
(12
): 1587-94
Nephropedia Template TP
gab.com Text
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English Wikipedia
Bing-Neel syndrome, a rare complication of Waldenström macroglobulinemia:
analysis of 44 cases and review of the literature A study on behalf of the
French Innovative Leukemia Organization (FILO)
#MMPMID26385211
Simon L
; Fitsiori A
; Lemal R
; Dupuis J
; Carpentier B
; Boudin L
; Corby A
; Aurran-Schleinitz T
; Gastaud L
; Talbot A
; Leprêtre S
; Mahe B
; Payet C
; Soussain C
; Bonnet C
; Vincent L
; Lissandre S
; Herbrecht R
; Kremer S
; Leblond V
; Fornecker LM
Haematologica
2015[Dec]; 100
(12
): 1587-94
PMID26385211
show ga
Central nervous system involvement by malignant cells is a rare complication of
Waldenström macroglobulinemia, and this clinicopathological entity is referred to
as the Bing-Neel syndrome. There is currently no consensus on the diagnostic
criteria, therapeutic approaches and response evaluation for this syndrome. In
this series, we retrospectively analyzed 44 French patients with Bing-Neel
syndrome. Bing-Neel syndrome was the first manifestation of Waldenström
macroglobulinemia in 36% of patients. When Waldenström macroglobulinemia was
diagnosed prior to Bing-Neel syndrome, the median time interval between this
diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study
highlights the possibility of the occurrence of Bing-Neel syndrome without any
other evidence of progression of Waldenström macroglobulinemia. The clinical
presentation was heterogeneous without any specific signs or symptoms.
Biologically, the median lymphocyte count in the cerebrospinal fluid was
31/mm(3). Magnetic resonance imaging revealed abnormalities in 78% of the cases.
The overall response rate after first-line treatment was 70%, and the overall
survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years.
Altogether, these results suggest that Bing-Neel syndrome should be considered in
the context of any unexplained neurological symptoms associated with Waldenström
macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid
analysis and magnetic resonance imaging of the brain and spinal axis. It still
remains difficult to establish treatment recommendations or prognostic factors in
the absence of large-scale, prospective, observational studies.
|*Central Nervous System Neoplasms/cerebrospinal
fluid/mortality/pathology/secondary/therapy
[MESH]