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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 Br+J+Haematol
2016 ; 172
(5
): 709-15
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Central nervous system involvement by Waldenström macroglobulinaemia (Bing-Neel
syndrome): a multi-institutional retrospective study
#MMPMID26686858
Castillo JJ
; D'Sa S
; Lunn MP
; Minnema MC
; Tedeschi A
; Lansigan F
; Palomba ML
; Varettoni M
; Garcia-Sanz R
; Nayak L
; Lee EQ
; Rinne ML
; Norden AD
; Ghobrial IM
; Treon SP
Br J Haematol
2016[Mar]; 172
(5
): 709-15
PMID26686858
show ga
Bing-Neel syndrome (BNS) is a rare complication seen in patients with Waldenström
macroglobulinaemia (WM), in which lymphoplasmacytic lymphoma cells colonize the
central nervous system. In this retrospective multi-centre study, we present the
clinicopathological features, imaging findings, therapy, response and outcomes of
34 patients with BNS. The median time from WM diagnosis to BNS diagnosis was 3
years, 15% of patients were diagnosed with BNS at the time of WM diagnosis, and
22% of patients developed BNS when responding to active treatment for WM.
Patients with BNS presented with variable clinical features including limb motor
deficits, change in mental status and cranial nerve palsies. The diagnosis was
made using a combination of cerebrospinal fluid cytology, flow cytometry and
detection of the MYD88 L265 mutation, and magnetic resonance imaging. The
estimated 3-year overall survival rate was 59%. Of the survivors, 40% have
evidence of pathological and/or radiological persistence of disease. Age older
than 65 years, platelet count lower than 100 × 10(9) /l, and treatment for WM
prior to BNS diagnosis were associated with worse outcome. Exposure to rituximab
for treatment of BNS was associated with a better outcome. Multi-institutional
collaboration is warranted to improve treatment and outcomes in patients with
BNS.
|Adult
[MESH]
|Aged
[MESH]
|Antineoplastic Agents/therapeutic use
[MESH]
|Central Nervous System Neoplasms/*diagnosis/drug therapy
[MESH]