Meningeal Lymphomatosis as the First Manifestation of Splenic Marginal Zone Lymphoma

Abstract

Meningeal lymphomatosis (ML) as the first manifestation of a splenic marginal zone lymphoma (SMZL) is rare. The descriptions of only 2 cases with this complication, one of which had ML as the first manifestation, have been published to date. We describe a 53-year-old man, an ex-smoker, who presented with transitory episodes of bilateral loss of visual acuity. On examination, only papilledema and splenomegalia were observed. The hemogram showed a predominance of lymphocytes with a villous morphology. Cytochemical staining and an immunophenotypic analysis revealed a positive reaction to tartrate-sensitive acid phosphatase and B-lineage markers (CD19+, CD20+, CD79b+, surface immunoglobulin 3 expression, immunoglobulin D+, CD5-, CD23-, CD10-, CD25-, CD103-, and CD11c-). Magnetic resonance imaging of the brain showed tumoral infiltration in both optic nerves and in the cervicodorsal meninges. The cerebrospinal fluid examination revealed significant pleocytosis, and all lymphocytes had a phenotype identical to that of the peripheral blood, confirming the presence of ML. The bone marrow section also showed lymphocytes with an immunophenotype identical to that of the peripheral blood.A splenectomy confirmed the SMZL diagnosis. Treatment with corticosteroids and intrathecal chemotherapy was administrated; however, the response was not good, and the patient died. In this report, we discuss the other 2 cases and ML in B-cell chronic lymphoproliferative disorders.

A Patient with Splenic Marginal Zone Lymphoma Presenting with Spastic Paraplegia as the Initial Symptom

“…Of the four previously reported cases of patients with SMZL, only two patients showed neurological symptoms as the initial manifestations [2, 3]. These two patients presented with leptomeningeal involvement, and the clinical manifestations of these two cases were polyradiculoneuropathy [2] and papilledema [3]. Our patient showed intramedullary spinal cord involvement as determined by MRI.…”

“…The CNS involvements in SMZL, as previously reported, were leptomeningeal involvement [2, 3, 4] and intracerebral tumor [5]. Of the four previously reported cases of patients with SMZL, only two patients showed neurological symptoms as the initial manifestations [2, 3]. These two patients presented with leptomeningeal involvement, and the clinical manifestations of these two cases were polyradiculoneuropathy [2] and papilledema [3].…”

“…Our patient presented with spastic paraplegia as the initial symptom. The CNS involvements in SMZL, as previously reported, were leptomeningeal involvement [2, 3, 4] and intracerebral tumor [5]. Of the four previously reported cases of patients with SMZL, only two patients showed neurological symptoms as the initial manifestations [2, 3].…”

“…Patients with this disease are typically elderly men, and this tumor usually involves the bone marrow, liver, and splenic hilar lymph nodes [1]. Involvement of the central nervous system (CNS) in the initial stage is rare in SMZL, and only a few cases of meningeal lymphomatosis as the first manifestation of SMZL have been reported in the literature [2, 3]. We report on the unique case of a patient with SMZL presenting with spastic paraplegia as the initial symptom.…”

B-cell lymphoma infiltration of cerebrospinal fluid with minimal symptoms and benign course

“…Meningeal lympho- matosis, as a manifestation of a MZL, has been described in only three patients. [5][6][7] Bruna et al 5 reported a patient with MZL infiltration in both optic nerves and in the meninges. Therapy with corticosteroids and intrathecal chemotherapy failed and the patient finally died.…”