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Haematology, Brains & Med. History - A case-based MCQ Quiz. #haematology #blooducation #FRCPath
A 75 yo man prev. treated for a haem malig. 2 yrs ago presents w/ 4 wks of unsteadiness. Now confused & disorientated.
If you could only have 1 of the following, which would it be?
A 75 yo man prev. treated for a haem malig. 2 yrs ago presents w/ 4 wks of unsteadiness. Now confused & disorientated.
If you could only have 1 of the following, which would it be?
You request his old haem notes, but in the mean time Neurology have scanned pt and performed diagnostic LP
MRI - leptomeningeal enhancement
CSF - monomorphic population of medium-sized mononuclear cells.
CD45+, CD19+, CD20+, CD79b+, CD52+, CD10-, BCL6-
These are...
MRI - leptomeningeal enhancement
CSF - monomorphic population of medium-sized mononuclear cells.
CD45+, CD19+, CD20+, CD79b+, CD52+, CD10-, BCL6-
These are...
Correct! There is a pop. of monoclonal mature B cells in the CSF (periph. blood contamination).
Time zones align & you get a collateral hx from his daughter in Oz. He was Rx'd for Waldenstroms 2 yrs ago with bendamustine.
Which mol. test could confirm these WM cells in CSF?
Time zones align & you get a collateral hx from his daughter in Oz. He was Rx'd for Waldenstroms 2 yrs ago with bendamustine.
Which mol. test could confirm these WM cells in CSF?
MYD88 mutation is detected, making it highly likely these are WM cells in the CSF. (MYD88 mut. present in >90% of WM cases).
What eponymous name is given to CNS Waldenstroms?
What eponymous name is given to CNS Waldenstroms?
Yes, this is Bing-Neel Syndrome. Named after Dr's Bing and Neel who reported two cases of neurological deficit in patients with hyperglobulinaemia. And this was in 1937, 8 years before Dr Waldenstrom described the primary condition!
In the same paper they called out BM biopsies as being a procedure likely to be of some use in the diagnosis of haematological conditions in the future..... Well, quite!
Back to the present. Our pt is unlucky enough to be one of the 1% of WM pts who develop Bing-Neel.
He is not fit for traditional intensive cytotoxic chemotherapies such as high-dose methotrexate.
Is there a newer agent we could offer, known to cross the blood-brain barrier?
He is not fit for traditional intensive cytotoxic chemotherapies such as high-dose methotrexate.
Is there a newer agent we could offer, known to cross the blood-brain barrier?
Ibrutinib is an established treatment for waldenstroms, and case reports in 2016 demonstrated that it effectively penetrates the CNS.
Since then a no. of papers have reported on ibrutinib used for Bing-Neel Syn, resulting in symptomatic and radiological improvement.
Since then a no. of papers have reported on ibrutinib used for Bing-Neel Syn, resulting in symptomatic and radiological improvement.
In Summary, Waldenstroms:
- Mature B lymphoplasmacytic cell neoplasm
- MYD88 mutation in >90%
- 1% of pt get CNS involvement
- This is called Bing-Neel Syn
- Gait abnormal. the most common symptom (50%)
- Rx'd with trad. CNS cytotoxic chemo
- Or ibrutinib as a new agent
- Mature B lymphoplasmacytic cell neoplasm
- MYD88 mutation in >90%
- 1% of pt get CNS involvement
- This is called Bing-Neel Syn
- Gait abnormal. the most common symptom (50%)
- Rx'd with trad. CNS cytotoxic chemo
- Or ibrutinib as a new agent
Thank you for reading - more about Waldenstroms at https://haembase.com/waldenstroms
Ref:
Bing & Neel 1936 - https://onlinelibrary.wiley.com/doi/abs/10.1111/j.0954-6820.1936.tb12571.x
Castillo & Treon 2019 - https://onlinelibrary.wiley.com/doi/pdf/10.1111/bjh.16167
Ref:
Bing & Neel 1936 - https://onlinelibrary.wiley.com/doi/abs/10.1111/j.0954-6820.1936.tb12571.x
Castillo & Treon 2019 - https://onlinelibrary.wiley.com/doi/pdf/10.1111/bjh.16167
