Primary CNS lymphoplasmacytic lymphoma: A case report and review of literature

Abstract

Lymphoplasmacytic lymphoma is a chronic lymphoproliferative disorder characterized by a proliferation of plasma cells, small lymphocytes, plasmacytoid lymphocytes and the production of monoclonal IgM. Primary central nervous system lymphomas (PCNSL) are rare non-Hodgkin lymphomas (NHL) that can be found in the brain, leptomeninges, eyes or spinal cord, and are mostly intracerebral. PCNSLs constitute 3-4% of primary brain tumors, and in most cases are diffuse large B-cell lymphomas (DLBCL).(1) Low grade lymphomas as primary central nervous system (CNS) lymphoma are very rare. We present here a case report of a woman who presented with headache and was found to have primary intracranial lymphoplasmacytic lymphoma (LPL).

Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome

“…Solitary BNS without concurrent or past WM has also been reported. 12 Asymptomatic BNS may exist (personal observation), but the incidence is unknown since CSF examination is not routinely carried out in WM.…”

“…The effective use of RT has been described in many case reports, both as first-line and as rescue therapy. 12,59,60 Localized RT to affected lesions at a dose of 30 to 40 Gy may be preferable to whole brain radiation to limit toxicity; this may fail to address widespread deposits that are not apparent on imaging. In general, cerebral RT, even when localized (stereotactic techniques), is associated with enhanced neurotoxicity, especially the occurrence of late neurocognitive effects in elderly patients which can affect up to 80% of patients.…”

Primary low-grade diffuse small lymphocytic lymphoma of the central nervous system

“…However, non-reactivity to CD-5 and CD-23 and positivity of plasma cells to CD-138, differentiates these lymphomas from SLL 14 15…”

Primary central nervous system germinal center B-like diffuse large B-cell lymphoma: a case report

“…PCNSL can be confused with multiple sclerosis (MS) due to clinical, radiological and histological similarities. Patients of PCNSL with the administration of corticosteroid present with clinical improvement and regression which may be interpreted as a steroid-induced remission from an exacerbation of MS (2). When the definite diagnosis was hardly obtained for those patients with lesions in the cortex or other sites which are easy to gain samples like our case, a biopsy is recommended as diagnostic routine (3).…”