scholarly journals CNS Vasculitis Associated with Waldenström Macroglobulinemia

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Tanawan Riangwiwat ◽  
Chris Y. Wu ◽  
Alberto S. Santos-Ocampo ◽  
Randal J. Liu ◽  
Aaron M. McMurtray ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Lymphoproliferative Disorder ◽  
Hyperviscosity Syndrome ◽  
Cns Vasculitis ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
Walking Aids ◽  
Intravenous Steroids

Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient’s mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis. To our knowledge, CNS vasculitis in a WM patient, in the absence of cryoglobulins, has not been reported. Immunosuppression is the preferred treatment.

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.

scholarly journals 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

Abstract 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.

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 Vasculitis on brain angiography is not always vasculitis: intravascular large B-cell lymphoma mimicking central nervous system vasculitis

2019 ◽  
Vol 12 (8) ◽  
pp. e230753
Author(s):  
Corbin Rayfield ◽  
Lester Mertz ◽  
Katalin Kelemen ◽  
Fawad Aslam
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
B Cell ◽  
Cell Lymphoma ◽  
Brain Biopsy ◽  
B Cell Lymphoma ◽  
Cns Vasculitis ◽  
Large B Cell Lymphoma ◽  
Angiographic Findings ◽  
Large B Cell

A 68-year-old man, with a history of non-Hodgkin’s lymphoma in remission, was admitted for homonymous hemianopsia, headaches and subacute progressive cognitive decline. Imaging revealed brain infarcts and angiography suggested vasculitis. A brain biopsy, however, revealed an intravascular large B-cell lymphoma (IVLBL). Central nervous system (CNS) vasculitis and IVLBL of the brain are extremely rare diseases that can have an almost identical clinical presentation. Angiographic findings are very similar but usually are reported as compatible with vasculitis. Brain biopsy or a random skin biopsy are crucial in diagnosing IVLBL as the accuracy of angiographic findings for CNS vasculitis is low.

scholarly journals “All the soarings of my mind begin in my blood:” central nervous system complication of Waldenström macroglobulinemia

2016 ◽  
Vol 91 (10) ◽  
pp. 1057-1060
Author(s):  
Seth N. Levin ◽  
Claudio M. de Gusmao ◽  
Mark R. Etherton ◽  
M. Will Rondeau ◽  
David M. Meredith ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Complication ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia
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