How we manage Bing-Neel syndrome.
Jorge J CastilloSteven P TreonPublished in: British journal of haematology (2019)
Bing-Neel syndrome (BNS) is an uncommon presentation of Waldenström macroglobulinaemia (WM), seen during the course of the disease in about 1% of patients. BNS occurs when WM cells gain access to the central nervous system (CNS) causing neurological deficits. The diagnosis of BNS is suggested by the presence of radiological abnormalities, such as leptomeningeal enhancement on magnetic resonance imaging and confirmed by the presence of clonal lymphoplasmacytic cells and MYD88 L265P in the cerebrospinal fluid. The treatment of BNS requires agents with good penetration into the CNS, such as fludarabine, methotrexate and cytarabine. The novel Bruton Tyrosine Kinase inhibitor ibrutinib has shown CNS-penetrating properties, and recent data suggest a therapeutic role in BNS. In this review, we will discuss the clinical and pathological features, diagnostic criteria, treatment options and outcomes of patients with BNS.
Keyphrases
- cerebrospinal fluid
- induced apoptosis
- magnetic resonance imaging
- cell cycle arrest
- blood brain barrier
- end stage renal disease
- newly diagnosed
- ejection fraction
- chronic kidney disease
- acute myeloid leukemia
- type diabetes
- signaling pathway
- endoplasmic reticulum stress
- prognostic factors
- small cell lung cancer
- big data
- adipose tissue
- pi k akt
- oxidative stress
- immune response
- electronic health record
- machine learning
- low dose
- glycemic control