Long Term Follow-Up of a Waldenström’s Macroglobulinemia Patient Presenting Bing-Neel Syndrome for the Last Eighteen Years - Abstract
Waldenström’s Macroglobulinemia (WM) is a rare lymphoproliferative disorder that presents a wide range of peculiar clinical manifestations; among them Bing-Neel Syndrome (BNS), concerning infiltration of the Central Nervous System (CNS), is extremely rare and was considered as carrying an adverse prognosis. We present here a 45-year-old woman diagnosed with WM that developed BNS while being in remission. She received rituximab intrathecally, producing complete resolution of leptomeningeal disease, lasting for 3 years. She subsequently relapsed several times locally and generally, developing lymphadenopathy and retroperitoneal mass that was also thought of adverse prognosis. She received various treatments and finally, in 2014, a Bruton’s tyrosine kinase inhibitor, Ibrutinib and achieved a remarkable improvement of her clinical status and a stability of imaging status, without entering complete remission although serum IgM is undetectable, and BM is disease free. She is still under the same treatment and in good clinical condition at present. Concluding, Bruton’s tyrosine kinase inhibitors very effectively control rare WM manifestations such as BNS and retroperitoneal disease.