scholarly journals Central Nervous System Involvement by Waldenstrom Macroglobulinemia: A Case Report of the Bing–Neel Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Ananth Arjunan ◽  
Hema Rai
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Standard Dose ◽  
Rare Complication ◽  
Central Nervous System Involvement ◽  
Serum Markers ◽  
High Dose ◽  
Neurologic Symptoms ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia

Bing–Neel syndrome (BNS) is a rare complication of Waldenstrom macroglobulinemia (WM) defined by a lymphoplasmacytic infiltration of the central nervous system (CNS). Patients present with a range of neurologic symptoms of variable severity. Diagnosis requires a low threshold of suspicion and is considered in WM patients with unexplained neurologic symptoms. It can occur in the presence of quiescent serum markers of WM. Direct CNS tissue biopsy should be pursued if feasible and remains the gold standard for diagnosis. No standard of care treatment exists, but expert guidelines suggest intravenous chemotherapy in standard dose or high-dose regimens or use of oral ibrutinib. Consideration is also made for intravenous rituximab, intrathecal therapies, and autologous stem cell transplantation. Patient factors and tolerability should drive decisions regarding treatment choice in this arena, given a lack of data for standard frontline therapy.

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

2021 ◽  
Vol 9 ◽  
pp. 232470962110212
Author(s):  
Hassaan Jafri ◽  
Isna Khan ◽  
Adarsh Sidda ◽  
Noman Ahmed Jang Khan ◽  
Murad Kheetan ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Kidney Function ◽  
Central Nervous System Involvement ◽  
Kidney Injury ◽  
Immunoglobulin M ◽  
Dramatic Improvement ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
The Central Nervous System

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.

scholarly journals Treatment of secondary central nervous system involvement in systemic aggressive B cell lymphoma using R-MIADD chemotherapy: a single-center study

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yuchen Wu ◽  
Xuefei Sun ◽  
Xueyan Bai ◽  
Jun Qian ◽  
Hong Zhu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Central Nervous System Lymphoma ◽  
Progression Free Survival ◽  
B Cell Lymphoma ◽  
High Dose ◽  
Cns Involvement ◽  
The Central Nervous System ◽  
Secondary Central Nervous System

Abstract Background Secondary central nervous system lymphoma (SCNSL) is defined as lymphoma involvement within the central nervous system (CNS) that originated elsewhere, or a CNS relapse of systemic lymphoma. Prognosis of SCNSL is poor and the most appropriate treatment is still undetermined. Methods We conducted a retrospective study to assess the feasibility of an R-MIADD (rituximab, high-dose methotrexate, ifosfamide, cytarabine, liposomal formulation of doxorubicin, and dexamethasone) regimen for SCNSL patients. Results Nineteen patients with newly diagnosed CNS lesions were selected, with a median age of 58 (range 20 to 72) years. Out of 19 patients, 11 (57.9%) achieved complete remission (CR) and 2 (10.5%) achieved partial remission (PR); the overall response rate was 68.4%. The median progression-free survival after CNS involvement was 28.0 months (95% confidence interval 11.0–44.9), and the median overall survival after CNS involvement was 34.5 months. Treatment-related death occurred in one patient (5.3%). Conclusions These single-centered data underscore the feasibility of an R-MIADD regimen as the induction therapy of SCNSL, further investigation is warranted.

Bing-Neel syndrome: A case reports

2020 ◽  
pp. 107815522098342
Author(s):  
Sinan Demircioğlu ◽  
Pembe Oltulu ◽  
Ganime D Emlik ◽  
Atakan Tekinalp ◽  
Özcan Çeneli
Keyword(s):  
Kinase Inhibitor ◽  
Case Reports ◽  
Rare Complication ◽  
Brain Biopsy ◽  
High Dose ◽  
Waldenström Macroglobulinemia ◽  
Dose Methotrexate ◽  
Waldenstrom Macroglobulinemia ◽  
Bruton Tyrosine Kinase ◽  
Methotrexate Treatment

Introduction Bing-Neel syndrome (BNS) is a rare complication of of Waldenström macroglobulinemia (WM) identified by involvement of central nervous system (CNS) lymphoplasmacytic cells. Case report We present a patient who was diagnosed with Bing-Neel syndrome four years after the diagnosis of Waldenström macroglobulinemia. Management & outcome The patient was admitted with neurological symptoms. There were lesions associated with WM involvement on brain imaging. The diagnosis was made by brain biopsy. High dose methotrexate treatment was given. Discussion CNS infiltrating agents such as fludarabine, methotrexate and cytarabine are often used in BNS treatment. Ibrutinib, which is a new bruton tyrosine kinase inhibitor, has recently started to be used in BNS treatment, as it has been shown to be effective and penetrate the CNS.

scholarly journals Immunogenicity of high-dose influenza vaccination in patients with primary central nervous system malignancy

2018 ◽  
Vol 5 (3) ◽  
pp. 176-183
Author(s):  
Roy E Strowd ◽  
Gregory Russell ◽  
Fang-Chi Hsu ◽  
Annette F Carter ◽  
Michael Chan ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Influenza Vaccine ◽  
Elderly Patients ◽  
Influenza Vaccination ◽  
Standard Dose ◽  
High Grade Glioma ◽  
Cns Lymphoma ◽  
High Dose ◽  
Central Nervous System Malignancy

Abstract Background For cancer patients, rates of influenza-associated hospitalization and death are 4 times greater than that of the general population. Previously, we reported reduced immunogenicity to the standard-dose influenza vaccine in patients with central nervous system malignancy. In other poorly responding populations (eg, elderly patients), high-dose vaccination has improved efficacy and immunogenicity. Methods A prospective cohort study was designed to evaluate the immunogenicity of the Fluzone® high-dose influenza vaccine in brain tumor patients. Data on diagnosis, active oncologic treatment, and immunologic status (eg, CD4 count, CD8 count, CD4:CD8 ratio) were collected. All patients received the high-dose vaccine (180 µg). Hemagglutination inhibition titers were measured at baseline, day 28, and 3 months following vaccination to determine seroconversion (≥4-fold rise) and seroprotection (titer ≥1:40), which were compared to our prior results. Results Twenty-seven patients enrolled. Diagnoses included high-grade glioma (85%), CNS lymphoma (11%), and meningioma (4%). Treatment at enrollment included glucocorticoids (n = 8, 30%), radiation (n = 2, 7%), and chemotherapy (n = 9, 33%). Posttreatment lymphopenia (PTL, CD4 ≤ 200) was observed in 4 patients (15%). High-dose vaccination was well tolerated with no grade III-IV toxicity. Overall, seroconversion rates for the A/H1N1, A/H3N2, and B vaccine strains were significantly higher than in our prior study: 65% vs 37%, 69% vs 23%, and 50% vs 23%, respectively (all P < .04). Seroconversion was universally poor in patients with PTL. While seroprotection at 3 months declined in our prior study, no drop was observed following high-dose vaccination in this cohort. Conclusions The immunologic response to HD influenza vaccination was higher in this cohort than standard-dose influenza vaccination in our prior report. These findings mirror those in elderly patients where high-dose vaccination is the standard of care and raise the possibility of an immunosenescence phenotype.

scholarly journals Ocular manifestations and treatment of central nervous system lymphomas

2006 ◽  
Vol 21 (5) ◽  
pp. 1-7 ◽  
Author(s):  
Kaan Gündüz ◽  
Jose S. Pulido ◽  
Colin A. McCannel ◽  
Brian Patrick O'Neill
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Systemic Chemotherapy ◽  
Central Nervous System Involvement ◽  
Central Nervous System Lymphoma ◽  
Tumor Formation ◽  
Lymphoid Cells ◽  
Current Status ◽  
Intraocular Lymphoma ◽  
High Dose

✓ Intraocular primary central nervous system lymphoma (PCNSL), also called primary intraocular lymphoma (PIOL), is a subset of PCNSL in which lymphoma cells invade the subretinal pigment epithelial space and vitreous cavity with or without central nervous system involvement at the time of ocular diagnosis. The frequency of this rare condition has increased over the past years in immunosuppressed as well as immunocompetent patients. The authors review the current status of PIOL and elaborate on their group's experience with its diagnosis and treatment. The incidence of PIOL is increasing. There is evidence that chronic antigenic stimulation may result in the development of PIOL. Recent advancements in the diagnosis of PIOL include better handling of vitreous specimens for cytological studies, immunocytological investigation for lymphoid cells, flow cytometry, cytokine evaluation, and molecular analysis. Because PIOL has a nonspecific presentation, the differential diagnosis should include infectious and noninfectious causes presenting with vitreitis and/or subepithelial infiltration as well as paraneoplastic syndromes including CRMP-5 optic neuropathies. Given that therapy is long-term and has significant systemic and ocular complications, tissue diagnosis is important. Treatment of PIOL may include systemic chemotherapy in which high-dose methotrexate-based regimens are used as well as intraocular injections of methotrexate and rituximab (anti-CD20 antibody). Cranial and ocular external-beam radiotherapy is being used less often today. Further studies are needed to prevent the tumor formation in terms of eliminating antigenic load and inhibiting B-cell chemokines as well as to determine the optimal local and systemic chemotherapy and immunotherapy options in the management of PIOL.

scholarly journals Report of 5 Cases of Extramedullary Myeloma with Central Nervous System Involvement Treated with a Combination of Carfilzomib/Thalidomide/Dexamethasone As a First Line Treatment at a Single Institution in Mexico

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5704-5704 ◽  
Author(s):  
Ramiro Espinoza ◽  
Diana Berenice Nolasco ◽  
Sosa Alejandro ◽  
Zapata Nidia ◽  
Cervera Eduardo ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Multiple Myeloma ◽  
Nervous System ◽  
Treatment Guidelines ◽  
Conflicts Of Interest ◽  
Central Nervous System Involvement ◽  
Infectious Complications ◽  
High Dose ◽  
Single Institution ◽  
Number Of Patients

Abstract Extramedullary myeloma (EMM) is defined by the presence of plasma cells outside the bone marrow in a patient with multiple myeloma (MM) (Touzeau & Moreau, 2016). EMM is by itself a rare entity with cases involving central nervous system (CNS) being rarer, it has an estimated incidence of 1-1.8% of all MM cases (Majd et al, 2015). Its presentation in comparison to classical MM has an adverse prognosis including a shorter progression free survival (PFS) (Gozzetti et al, 2015) and overall survival (OS) with a mean life expectancy of 1.5-6 months (Majd et al, 2015). Currently there are not international treatment guidelines for this disease. The aim of this report is to illustrate our experience at a single institution in Mexico, presenting five cases of CNS-EMM treated with proteasome inhibitor (carfilzomib) in combination with thalidomide, dexamethasone plus radiotherapy. The patient's characteristics and the treatment regimen are shown in table 1 and 2. Within three months, following the criteria to the international multiple myeloma working group (IMMWG), all five patients accomplished a positive response (one partial response [PR] four very good partial responses [VGPR]), at sixth months two patients improved their response from previously PR to a VGPR and from VGPR to complete response (CR), one sustained its VGPR and after this period of time two patients had progressive disease (PD) and died due to infectious complications. After the tenth month a third patient also died of infectious complications. By the end of this report two patients still alive, one is being evaluated for autologous stem cell transplantation (ASCT) and the other one wasn't eligible according to European Group for Blood and Marrow Transplantation (EBMT) risk score and will continue treatment without ASCT. Previously reported cases in the literature with old chemotherapy regimens shown to have median survival of 1.5 months (Petersen et al 1999), nowadays the recommended therapy is based on triplet induction therapy followed by high-dose melphalan/ASCT, a triplet consolidation therapy and maintenance treatment with lenalidomide (Touzeau & Moreau, 2016); so far there is no evidence of proteasome inhibitors penetrating the blood brain barrier (Nooka et al, 2013) and this might be the reason why the literature strongly recommends the use of lenalidomide as this one does penetrate into the CSF after oral administration (Muscal et al, 2012), we did not use lenalidomide due to the high monetary cost of the treatment but instead we use thalidomide in combination with carfilzomib and dexamethasone with improved OS in three of our five patients (VGPR + CR). Despite the small number of patients presented in this report and the lack of cytogenetics or FISH information, we think this might be a good therapeutic approached in patients with CNS-EMM, especially in those scenarios with not accessible resources or absence of clinical trials. Further studies evaluating the addition of monoclonal antibodies (daratumumab, elotuzumab) need to be done in order to improve the OS and PFS in this group of patients. Disclosures No relevant conflicts of interest to declare.

Blood viscosity in Waldenstrom macroglobulinemia

Blood ◽  
1977 ◽  
Vol 49 (4) ◽  
pp. 507-510 ◽  
Author(s):  
MR MacKenzie ◽  
TK Lee
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Direct Measurement ◽  
Blood Viscosity ◽  
Whole Blood ◽  
Hyperviscosity Syndrome ◽  
Whole Blood Viscosity ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
Low Serum

Patients with Waldenstrom macroglobulinemia were studied for the presence or absence of the hyperviscosity syndrome, the relative serum viscosity value, and the calculated whole blood viscosity to identify a level at which symptoms occurred. The majority of symptomatic patients had whole blood viscosity values above 8.0 centipoises. There was a direct correlation between whole blood viscosity and relative serum viscosity, r = 0.75. One patient with central nervous system abnormalities was identified as having a high whole blood viscosity but a low serum viscosity. It was concluded that the vast majority of patients with the hyperviscosity syndrome will be identified by measuring the relative serum viscosity. In patients with central nervous system findings and a low serum viscosity, the whole blood viscosity should be determined either by direct measurement or by calculation.

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