Bing–Neel syndrome (BNS) is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM), which is a chronic lymphoproliferative disorder.
[1] There's no clear definition of BNS but what is known so far is that unlike WM, It involves the central nervous system (CNS), infiltrated by differentiated malignant B cells and by having hyperglobulinemia.
Some sensory symptoms include a pin and needles sensation experienced in the lower limbs, the hands, and in the arms, along with pain and extreme numbness.
[citation needed] Infiltration of malignant, differentiated B-cells linked to WM into the nervous system precipitates BNS.
Histological practices that entail a biopsy of the cerebrum and/or the meninges look for the presence of lymphoplasmacytic lymphomas (Mature B-cells).
[3][4] Analysis entails analyzing several different aspects of the cerebrospinal fluid (CSF) to identify characteristics linked to WM and BNS.
[citation needed]Flow cytometry, used to identify cell biomarkers, is an auxiliary tool used in CSF analysis.
[3][4][5] MRI with gadolinium contrast is the primary radiologic tool used to diagnose ailments of the central nervous system, BNS included.
During CSF analysis, PCR amplification of genomic DNA found in the fluid, followed by sequencing, can determine if the mutation is present within the CNS; if so, this would be indicative of, though not conclusive, of BNS.
[4] There are a few options when it comes to treatment so the type one will choose is completely individualized, taking into consideration the person's state or condition and liking.
[4][7] Bruton's Tyrosine Kinase Inhibitors are very active in Bing Neel Syndrome and have largely become the standard for frontline treatment of this manifestation of Waldenstrom's Macroglobulinemia.
Out of 5 patients with repeated CSF analysis, 3 demonstrated persistent detectable clonal LPL cells.
[4] Bing–Neel syndrome was first described by Jens Bing and Axel Valdemar Neel, who observed a case of 2 women, 56 and 39 years old, presenting with rapid neurodegeneration in the setting of hyperglobulinemia.
It was only 80 years later that there was a meeting with a group of people at the 8th International Workshop on WM to come up with broad diagnostic criteria for BNS.
This group of people included radiologists, immunologists, hematologists, and neurologists from all over the world using PubMed as their source for the guideline.