High‐dose therapy with autologous stem cells transplantation in Bing‐Neel syndrome: A retrospective analysis of 14 cases

Simon, Laurence; Fornecker, Luc‐Matthieu; Tournilhac, Olivier; Leblond, Véronique

doi:10.1002/ajh.25547

Cited by 9 publications

(6 citation statements)

References 10 publications

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“…The literature data on auto-HCT in BNS are extremely scarce. Simon et al summarized the French experience with auto-HCT in BNS, covering solely 14 patients transplanted between 1999–2018 [ 11 ]. In their analysis, auto-HCT was used as part of the first-line strategy in 11 patients, and salvage treatment—in the further 3 patients.…”

Section: Discussionmentioning

confidence: 99%

Bing-Neel Syndrome, a Rare Presentation of Waldenström Macroglobulinemia—A Multicenter Report by the Polish Lymphoma Research Group

Drozd-Sokołowska

Waszczuk‐Gajda

Witkowska

et al. 2022

JCM

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Bing-Neel syndrome (BNS) is a rare presentation of Waldenström macroglobulinemia (WM). BNS is a consequence of the central nervous system (CNS) involvement by lymphoplasmacytic lymphoma (LPL) and, rarely, the peripheral nervous system. The data on BNS are extremely scarce. Therefore, we performed a multicenter retrospective analysis of BNS patients diagnosed and treated in centers aligned with the Polish Lymphoma Research Group. The analysis covers the years 2014–2021. Eleven patients were included, 55% females and the median age at BNS diagnosis was 61 years. The median time from WM to BNS was 3.5 years; 27% of patients did have a diagnosis of WM and BNS made simultaneously or within 30 days from each other. Isolated parenchymal involvement was the least frequent (20%). Patients were treated with different regimens, mostly able to cross the blood-brain barrier, including 18% treated with ibrutinib first line. The cumulative objective response to treatment was 73%. With the median follow-up of 20 months (95% CI, 2–32), the 36-month estimates were: overall survival (OS) 47%, progression-free survival (PFS) 33%, and cumulative incidence of BNS-associated death 41%. The performance status according to ECOG was significant for PFS (HR = 7.79) and the hemoglobin concentration below 11 g/dL was correlated with PFS. To conclude, BNS is a very rare manifestation of WM. It is associated with a poor outcome with most patients succumbing to BNS.

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Section: Discussionmentioning

confidence: 99%

Bing-Neel Syndrome, a Rare Presentation of Waldenström Macroglobulinemia—A Multicenter Report by the Polish Lymphoma Research Group

Drozd-Sokołowska

Waszczuk‐Gajda

Witkowska

et al. 2022

JCM

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“…Second-generation BTK inhibitors have demonstrated safety and efficacy for WM patients [ 13 - 14 ] and may prove useful in treating BNS as well. Finally, autologous stem cell transplant has achieved remarkable results in a small case series, where 11/14 patients with BNS showed disease response and 10/14 remained in remission at three years [ 15 ]. The prognosis of BNS remains unclear; a 34-patient series estimated three-year survival at 59% [ 4 ], whereas a 44-patient series estimated five-year survival at 71% [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

Bing-Neel Syndrome: An Initial Manifestation of Waldenstrom Macroglobulinemia

Lee¹,

S²,

Valasapalli³

et al. 2021

Cureus

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Waldenstrom macroglobulinemia (WM) is a low-grade B-cell lymphoma characterized by bone marrow infiltration by monoclonal lymphoplasmacytic cells plus an IgM monoclonal gammopathy. Bing-Neel syndrome (BNS) is a rare manifestation of WM where malignant lymphoplasmacytic cells infiltrate the central nervous system (CNS). Though only present in 0.8% of WM cases, it is likely underdiagnosed and may present before or during WM treatment. Here, we present a case of BNS as an initial sign of WM. A 75-year-old male presented with confusion, gait instability, and expressive aphasia. MRI demonstrated a 5.5-cm mass in the right frontal lobe, crossing midline. Brain biopsy showed CNS lymphoma and later tested positive for the MYD88L265P mutation suggesting WM (as is a mutation in 90-95% WM patients). Indeed, quantitative serum immunoglobulins showed elevated IgM. Initial treatment for WM was started with rituximab, methylprednisolone, carfilzomib, and ibrutinib. MRI two months after initiation showed good response, and the patient was transitioned to ibrutinib monotherapy. Surveillance MRI one year later showed patchy right frontal lobe enhancement indicating disease progression, and therefore the patient was placed back on his initial treatment regimen. However, ibrutinib later had to be held due to thrombocytopenia. Two months after re-starting chemotherapy, he presented with bizarre behavior, and MRI showed extensive disease progression. He was then transitioned to palliative chemotherapy with high-dose methotrexate and rituximab. He has responded well to this regimen, and MRI two years after diagnosis showed no recurrent disease. BNS is a rare but easily missed manifestation of WM. As per the recent National Comprehensive Cancer Network (NCCN) guidelines and the 8th International Workshop on WM (IWWM-8), no standardized diagnostic or management guidelines for BNS is available. Direct brain biopsy is the gold standard for diagnosis. Due to its low incidence, rarity, and limited prospective trial, there is a lack of a clear standard of care therapy. Specific treatment regimen depends on the patient factors and treatment tolerability. IWWM-8 suggests the use of a variety of cytotoxic chemotherapies or ibrutinib. A high-quality meta-analysis of existing reports is critical to characterize the diagnostic features and optimal treatment for BNS. The prognosis of BNS remains unclear, with an estimated three- and five-year survival rate at 59% and 71%, respectively. BNS is an infrequent complication of WM. Clinicians should suspect BNS with persistent, unexplained neurologic symptoms in WM.

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“…Systemic chemotherapy and radiotherapy are considered in group A patients in comparison to those in group B with low cellular infiltration and accompanying symptoms of IgM deposition, where the therapeutic approach may include plasmapheresis. Autologous transplantation of hematopoietic stem cells has been applied in selected patients [19]. Novel agents such as ibrutinib -an inhibitor of Bruton tyrosine kinase (BTK), has demonstrated high response rates [20].…”

Section: Discussionmentioning

confidence: 99%

Bing-Neel Syndrome – A Challenging Diagnosis: Case Report

Davidkova

Simonoski

Spassov

et al. 2020

IBRR

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Bing-Neel syndrome is a rare neurologic complication of Waldenström’s macroglobulinemia, characterized by infiltration of the central nervous system by clonal lymphoplasmacytes. We present a rare clinical case of a patient, who one year after the diagnosis of Waldenström’s macroglobulinemia, progressed with diverse neurologic presentation and cerebrospinal fluid involvement. The diagnosis was based on magnetic resonance imaging and flow cytometry detection of clonal B-cells in the cerebrospinal fluid. Bing-Neel syndrome should be considered in patients with neurologic symptoms and a history of Waldenström’s macroglobulinemia.

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High‐dose therapy with autologous stem cells transplantation in Bing‐Neel syndrome: A retrospective analysis of 14 cases

Cited by 9 publications

References 10 publications

Bing-Neel Syndrome, a Rare Presentation of Waldenström Macroglobulinemia—A Multicenter Report by the Polish Lymphoma Research Group

Bing-Neel Syndrome, a Rare Presentation of Waldenström Macroglobulinemia—A Multicenter Report by the Polish Lymphoma Research Group

Bing-Neel Syndrome: An Initial Manifestation of Waldenstrom Macroglobulinemia

Bing-Neel Syndrome – A Challenging Diagnosis: Case Report

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