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 ◽  
...  
2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Ananth Arjunan ◽  
Hema Rai

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.


Blood ◽  
1977 ◽  
Vol 49 (4) ◽  
pp. 507-510 ◽  
Author(s):  
MR MacKenzie ◽  
TK Lee

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.


2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Tanawan Riangwiwat ◽  
Chris Y. Wu ◽  
Alberto S. Santos-Ocampo ◽  
Randal J. Liu ◽  
Aaron M. McMurtray ◽  
...  

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.


2021 ◽  
Vol 9 ◽  
pp. 232470962110212
Author(s):  
Hassaan Jafri ◽  
Isna Khan ◽  
Adarsh Sidda ◽  
Noman Ahmed Jang Khan ◽  
Murad Kheetan ◽  
...  

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.


Blood ◽  
1977 ◽  
Vol 49 (4) ◽  
pp. 507-510 ◽  
Author(s):  
MR MacKenzie ◽  
TK Lee

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.


2021 ◽  
Vol 19 ◽  
pp. 205873922110005
Author(s):  
Bei Lu ◽  
Yang Cai ◽  
Junjie Yin ◽  
Jingrui Wang ◽  
Zhong Jia ◽  
...  

Patients with acute pancreatitis (AP) often suffer tough complications, some of which are fatal. The early diagnosis and definite treatment of central nervous system (CNS) complications have not been fully achieved yet, which seriously affects the mortality of severe acute pancreatitis (SAP). We present a case of infected pancreatic necrosis (IPN) in a 62-year Chinese man who developed acute herpes simplex encephalitis (HSE) caused by herpes simplex virus type 1 (HSV-1) after favorable minimally invasive retroperitoneal approaches (MIRAs). The patient was successfully treated with 115 days stayed in our hospital. The MIRAs included image-guided retroperitoneal percutaneous catheter drainage (PCD), nephroscopic pancreatic necrosectomy (NPN), and ultrasonic pneumatic lithotripsy system (UPLS) assisted non-narcotic sinus track necrosectomy (NSN). HSE is relatively rare and potentially life threatening. We attempt to discuss the probable risk factors and how the relatively rare HSE are related to the patients of SAP with latent HSV.


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