scholarly journals Development of Central Nervous System Vasculitis in a Patient with Waldenstrom Macroglobulinemia: A Rare Presentation with Poor Prognosis

Cureus ◽  
2019 ◽  
Author(s):  
Tahir Muhammad Abdullah Khan ◽  
Yusra Ansari ◽  
Abdul Hasan Siddiqui ◽  
Saad Ali Ansari ◽  
Faraz Siddiqui
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Poor Prognosis ◽  
Central Nervous System Vasculitis ◽  
Rare Presentation ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia

scholarly journals Central Nervous System Vasculitis as a Rare Presentation of Mycoplasma pneumoniae: A Case Report

2020 ◽  
Vol 12 (3) ◽  
pp. 402-409
Author(s):  
Ashraf Omer Elamin Ahmed ◽  
Mona Mohammad Ibraheem Babikir ◽  
Amir Elssoni Mahjoup Khojali ◽  
Suresh Nalaka Menik Arachchige ◽  
Abdirahman Mohamud Abdirahman ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Lower Respiratory Tract ◽  
Respiratory Tract Infections ◽  
Central Nervous System Involvement ◽  
Lower Respiratory Tract Infections ◽  
Central Nervous System Vasculitis ◽  
Rare Presentation ◽  
Systemic Involvement ◽  
Tract Infections

<i>Mycobacteria pneumoniae</i> (MP) commonly causes upper and lower respiratory tract infections. The clinical manifestation is classified as pulmonary and extrapulmonary. These manifestations vary according to the involved system. MP may affect one system or more at a time. Commonly prodromal respiratory symptoms precede systemic involvement. Central nervous system involvement in uncommon. This report is presenting a rare case of central nervous system vasculitis secondary to MP, highlighting the diagnosis and management with a succinct literature review.

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.

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.

Rare Presentation of a Relapsing Remitting Central Nervous System Vasculitis: A Case Report.

2019 ◽  
Vol 87 (02) ◽  
pp. 129-132
Author(s):  
Maksymilian Brzezicki ◽  
Matthew Kobetic ◽  
Dillon Serena
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Central Nervous System Vasculitis ◽  
Rare Presentation ◽  
Relapsing Remitting

ZusammenfassungWir berichten über einen interessanten Fall einer Vaskulitis des zentralen Nervensystems, der eine schubförmig-remittierende Natur und ein bisher nicht berichtetes Profil kognitiver Defizite bei dieser Variante der Erkrankung aufweist.Der Patient stellte sich mit beidseitigem Kopfschmerz, Gesichtsfeldverlust des linken Auges, Dyschromatopsie und vorübergehender Bewusstlosigkeit vor. Er wurde in den letzten acht Jahren auf ähnliche Symptome untersucht. Es gab eine klare schubförmig-remittierende Geschichte, mit Perioden voller Remission von 4-6 Monaten.MRT-Untersuchungen zeigten bilaterale Bereiche von Hyperintensitäten, repräsentativ für kleine „nicht-charakteristische“ Veränderungen, wahrscheinlich vaskulären Ursprungs.In diesem Bericht wird die klinische Konundra während der Evaluation des Patienten diskutiert, mögliche Differentialdiagnosen untersucht und über die hypothetische Rolle der Statinverwendung in ähnlichen Fällen kommentiert.

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.

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 Anti-neutrophil cytoplasmic antibody–associated vasculitis complicated with diffuse alveolar haemorrhage and central nervous system vasculitis

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110118
Author(s):  
Takashi Nawata ◽  
Kaori Murakawa ◽  
Kosaku Shiragami ◽  
Masaki Shibuya ◽  
Makoto Kubo ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Systemic Vasculitis ◽  
Alveolar Haemorrhage ◽  
Simultaneous Occurrence ◽  
Cerebral Stroke ◽  
Diffuse Alveolar Haemorrhage ◽  
Central Nervous System Vasculitis ◽  
Rare Presentation ◽  
Life Threatening

Diffuse alveolar haemorrhage and central nervous system vasculitis are life-threatening complications of anti-neutrophil cytoplasmic antibody-associated vasculitis. The simultaneous occurrence of diffuse alveolar haemorrhage and central nervous system vasculitis is a rare presentation of antibody-associated vasculitis. Its diagnosis by histopathology is difficult because biopsy is difficult to perform, and urgent treatment is needed. We report a case of a Japanese man with diffuse alveolar haemorrhage and central nervous system vasculitis associated with antibody-associated vasculitis. New classification criteria may be needed for diffuse alveolar haemorrhage and central nervous system vasculitis associated with systemic vasculitis. When antibiotic-resistant atypical bilateral pneumonia is noted in the acute phase of a cerebral stroke, with elements suggestive of vasculitis, clinicians should be aware that diffuse alveolar haemorrhage and central nervous system vasculitis may occur simultaneously.

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.

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