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Bing and neel syndrome.

S Jennane¹, K Doghmi, E M Mahtat

¹Service d'Hématologie Clinique, Hôpital Militaire d'Instruction Mohammed V, Rabat, Morocco.

Case Reports in Hematology

|September 19, 2012

Summary

Bing and Neel syndrome (BNS) is a rare complication of Waldenström's macroglobulinemia (WM). This case highlights BNS presenting as isolated ptosis, emphasizing its potential treatability in WM patients with neurological symptoms.

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In Vivo Functional Study of Disease-associated Rare Human Variants Using Drosophila

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Area of Science:

Neurology
Hematology
Oncology

Background:

Waldenström's macroglobulinemia (WM) is a rare lymphoproliferative disorder.
Bing and Neel syndrome (BNS) is a rare neurological complication of WM.
BNS can manifest with various neurological symptoms, including ptosis.

Purpose of the Study:

To report a case of Bing and Neel syndrome (BNS) revealed by isolated left ptosis in a patient with Waldenström's macroglobulinemia (WM).
To discuss the diagnostic approach and treatment strategies for BNS in the context of WM.
To highlight the importance of considering BNS in WM patients presenting with neurological deficits.

Main Methods:

Case report of a 57-year-old male patient with a history of WM.
Diagnosis of BNS based on clinical presentation (isolated left ptosis), MRI findings, and detection of monoclonal IgM in cerebrospinal fluid (CSF).
Treatment involved chemotherapy regimens (RDHAP, RDHOx), intra-CSF chemotherapy, and autologous stem cell transplantation.

Main Results:

The patient presented with isolated left ptosis, which led to the diagnosis of BNS.
A partial remission of BNS was achieved with multi-agent chemotherapy and intra-CSF injections.
Despite treatment intensification and stem cell transplantation, the patient succumbed to septic shock post-transplant.

Conclusions:

Bing and Neel syndrome is a rare but potentially treatable complication of Waldenström's macroglobulinemia.
Neurological symptoms, such as isolated ptosis, in WM patients warrant investigation for BNS.
Early diagnosis and appropriate treatment may improve outcomes, although BNS can be aggressive.