How we manage Bing–Neel syndrome

Castillo, Jorge J.; Treon, Steven P.

doi:10.1111/bjh.16167

Cited by 54 publications

(70 citation statements)

References 65 publications

(91 reference statements)

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“…Rituximab monotherapy is not recommended in BNS because of limited CNS penetration but can be used in conjunction with other therapies for systemic control of disease. 12 In 2015, ibrutinib was first approved for symptomatic WM by study done by Treon et al where ibrutinib was given to patients with WM in second-line setting. Patients with BNS were not included.…”

Section: Discussionmentioning

confidence: 99%

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Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib

Jafri

Khan

Sidda

et al. 2021

Journal of Investigative Medicine High Impact Case Reports

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Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma associated with a monoclonal immunoglobulin M protein. Extranodal involvement in WM is not very common. In this article, we present a rare case of WM with kidney and central nervous system involvement. Bing-Neel syndrome is a distinct complication of WM where lymphoplasmacytic cells involve the central nervous system (CNS). Our patient was initially treated with dialysis and steroids with improvement in his kidney function. He was then started on systemic treatment with rituximab, cyclophosphamide, and dexamethasone with stable kidney function but persistent CNS symptoms. Due to rarity of cases, there is no standard treatment for Bing-Neel syndrome. His treatment was switched to ibrutinib with dramatic improvement in his CNS symptoms as well as radiological findings on magnetic resonance imaging.

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Section: Discussionmentioning

confidence: 99%

“…Rituximab monotherapy is not recommended in BNS because of limited CNS penetration but can be used in conjunction with other therapies for systemic control of disease. 12 …”

Section: Discussionmentioning

confidence: 99%

Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib

Jafri

Khan

Sidda

et al. 2021

Journal of Investigative Medicine High Impact Case Reports

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“…CSF should be evaluated by morphology, flow cytometry and molecular studies. 23 Flow cytometry of CSF is the most sensitive technique to detect tumor cells in the central nervous system, 24 but PCR assays to detect IGH gene rearrangements and mutated MYD88 L265P on CSF can also help to support the diagnosis of BNS. In patients with focal brain lesions but no CSF involvement, a stereotaxic biopsy should be programmed.…”

Section: Extra-medullary Disease (Emd)mentioning

confidence: 99%

“…In patients with focal brain lesions but no CSF involvement, a stereotaxic biopsy should be programmed. 23…”

Section: Extra-medullary Disease (Emd)mentioning

confidence: 99%

Waldenström’s Macroglobulinemia: An Exploration into the Pathology and Diagnosis of a Complex B-Cell Malignancy

Askari

Rodríguez

2021

JBM

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After 77 years since the initial description, Waldenström macroglobulinemia (WM) remains as a bone marrow neoplastic disorder with lymphoplasmacytic differentiation oversecreting a monoclonal immunoglobulin M (IgM). However, many biological and genetic aspects of this entity have been unraveled and it is now easy to correctly diagnose patients with this illness. The diagnosis requires the presence of a monoclonal IgM component and bone marrow lymphoid infiltration must be demonstrated. In addition, other small B-cell lymphoid neoplasms with plasma cell differentiation must be discarded. Although the clinical picture is highly heterogeneous, the diagnosis is much easier today compared to the past, since now we can demonstrate the presence of somatic mutations, especially the L265P mutation in the MYD88 gene, highly characteristic of WM (>90% of the patients), followed by the WHIM-like mutations in the CXCR4 gene (~35%). The identification of these mutations is very important, because they can modulate the response to new treatments with Bruton's tyrosine kinase (BTK) inhibitors. Thus, the conventional prognostic factors that predict the outcome of these patients (anemia, thrombopenia, high M component, high B2M, and advanced age), must be complemented with the genetic evaluation of the patient, that can help us in the prediction of the risk of transformation from asymptomatic to symptomatic forms (Del6q) and/or from indolent forms of the disease to aggressive lymphomas (CD79b mutations).

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“…A neurological examination is necessary for the documentation and characterisation of peripheral neuropathy. The presence of neurological symptoms suggesting central nervous system pathology in the context of WM should raise the suspicion of Bing Neel syndrome [ 55 ].…”

Section: Approach To the Clinical Evaluation And Initial Investigamentioning

confidence: 99%

Immunoglobulin M Paraproteinaemias

Girard

Soekojo

Ooi

et al. 2020

Cancers

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Monoclonal paraproteinaemia is an increasingly common reason for referral to haematology services. Paraproteinaemias may be associated with life-threatening haematologic malignancies but can also be an incidental finding requiring only observation. Immunoglobulin M (IgM) paraproteinaemias comprise 15–20% of monoclonal proteins but pose unique clinical challenges. IgM paraproteins are more commonly associated with lymphoplasmacytic lymphoma than multiple myeloma and can occur in a variety of other mature B-cell neoplasms. The large molecular weight of the IgM multimer leads to a spectrum of clinical manifestations more commonly seen with IgM paraproteins than others. The differential diagnosis of B-cell and plasma cell dyscrasias associated with IgM gammopathies can be challenging. Although the discovery of MYD88 L265P and other mutations has shed light on the molecular biology of IgM paraproteinaemias, clinical and histopathologic findings still play a vital role in the diagnostic process. IgM secreting clones are also associated with a number of “monoclonal gammopathy of clinical significance” entities. These disorders pose a novel challenge from both a diagnostic and therapeutic perspective. In this review we provide a clinical overview of IgM paraproteinaemias while discussing the key advances which may affect how we manage these patients in the future.

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How we manage Bing–Neel syndrome

Cited by 54 publications

References 65 publications

Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib

Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib

Waldenström’s Macroglobulinemia: An Exploration into the Pathology and Diagnosis of a Complex B-Cell Malignancy

Immunoglobulin M Paraproteinaemias

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