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Here we present a diagnostically challenging Bing-Neel Syndrome (BNS) case successfully treated with systemic chemoimmunotherapy and ibrutinib, with remarkable clinical response. BNS is an extremely rare direct central nervous system infiltration by malignant lymphoplasmocytic lymphoma cells in patients with Waldenstroms macroglobulinaemia.

Key Clinical Message We present a case of Bing–Neel syndrome a rare central nervous system lymphoplasmocytic lymphoma associated with Waldenstrom macroglobulinemia. Diagnosis should be considered in the context of unexplained neurological symptoms in the presence of macroglobulinemia. Abstract Waldenstroms macroglobulinaemia (WM) is a rare B‐cell lymphoma representing ~2% of all hematological malignancies. While most neurological complications of WM are secondary to the overproduction of immunoglobulin M (IgM), Bing‐Neel syndrome (BNS) is an extremely rare direct central nervous system (CNS) infiltration by malignant lymphoplasmocytic lymphoma (LPL) cells. Limited information on BNS exists in the literature with sparse case reports and case series. Here, we present a diagnostically challenging BNS case successfully treated with systemic chemoimmunotherapy and ibrutinib, with remarkable clinical response.

Nodal peripheral T cell lymphoma (PTCL) with T follicular helper (TFH) cell phenotype is a provisional entity added to the 2016 revised WHO classification of haematological malignancies. These lymphomas have an aggressive clinical course and respond poorly to conventional treatments. Distinct histological features have not been well described. Additionally, the minimum criteria for diagnosis is not well established but detection of at least two TFH markers in addition to CD4 is suggested to assign a TFH cell phenotype. Some pathological features of angioimmunoblastic T cell lymphoma (AITL) such as recurrent molecular alterations are commonly found. As the name suggests, these lymphomas are nodal in origin with patients presenting with widespread lymphadenopathy. We describe the first documented case of nodal PTCL with a TFH phenotype presenting as an isolated mesenteric mass with no nodal involvement.