Monoclonal gammopathy as a marker of primary cerebral lymphoma

Sir, In this report, we describe two patients with PCNSL marked by an MC with no signs of immunodeficiency or other haematologic alteration. The first was a 79-year-old woman who came for our observation in 2002 because of a serum monoclonal IgGj of 1Æ8 g dL (Fig. 1a,b). Clinical and routine blood exams were normal. No bone marrow infiltration was observed, and no urinary MC was detected with immunofixation. No evidence of osteolysis or spinal involvement was present. After 2 months, decrease in visus and amaurosis at left eye was observed. Patient underwent CNS magnetic resonance (MRI) which evidenced an expansive process at the frontal lobe (Fig. 1c). After neurosurgical removal, the mass was histologically classified as a follicular non-Hodgkin’s lymphoma CD20 ⁄ CD45 ⁄ j chain. Neither systemic lymphadenopathy nor liver ⁄ spleen infiltration was found. After surgery, the MC disappeared (Fig. 1d,e) and chemotherapy with Rituximab-CyclophosphamideAdriamycin-Vincristine-Prednison (R-CHOP) was started. After a 6-year follow-up, no evidence of relapse or recurrence of the MC has been observed. In the second case, a 52-year-old woman came for neurological observation in August 2004 because of headache, asthenia, vertigo and episodic dyplopia. A serum monoclonal IgAj of 1Æ4 g dL was found. In the above described patient, no systemic disease was evidenced. In January 2005, the patient came for our observation: the laboratory tests remained unchanged from the previous ones. At the bone marrow biopsy, an infiltration of CD138 ⁄ CD20 ⁄ j chain lymphoplasmacytes was found. No systemic lymphadenopathies or liver ⁄ spleen infiltration was observed. The CNS MRI revealed an expansive process in the tuberculum sellae. After neurosurgical removal, it was classified as a lymphoplasmacytic non-Hodgkin’s lymphoma CD138 ⁄ CD20 ⁄ CD5 ⁄ CD45 ⁄ j chain ⁄ k chain(Immunocytoma) and MC disappeared. The patient was treated with R-CHOP and intratecal administration of methotrexate. In August 2006, the serum MC reappeared, lytic lesions at spine, skull and femur were observed and marrow lymphoplasmacytes rose to 20%. The patient underwent savage therapy, with regression of disease and serum MC disappeared. Primary central nervous system lymphoma (PCNSL) is a rare disease, accounting for a few percentage of intracranial tumours [1] that recur mostly in immunodeficient subjects [2]. CNS localization recurs in 5–30% of other diseases such as systemic NHLs, in which a serum monoclonal component (MC) may represent a marker of the former [1]. To our knowledge, no association of MC and PCNSL has been reported, all the described cases being atypical localization of systemic disease [3–6]. Treatment of PCNSLs is still undetermined. Combined treatments (chemo-radiotherapy, surgery plus chemo-radiotherapy, intratecal chemotherapy plus radiotherapy or surgery or systemic chemotherapy) have given better results than a single treatment [1]. In most patients who survive longer than 2 years, PCNSL recurs and the relapse can be evidenced only by means of imaging study. There are no useful serum markers during the follow-up. In our patients, the periodic evaluation of the serum MC was a key marker because of the immediate evidence of disease recurrence and systemic diffusion in the second patient, but not in the first patient in whom its negativity revealed absence of recurrence at brain level and at distant sites.