Mantle cell lymphoma: a retrospective study of 121 cases

Abstract

Mantle cell lymphoma (MCL) patients represent a difficult problem, sometimes to establish the diagnosis but mostly because of their refractoriness to standard lymphoma treatments. Which treatments to apply and to whom is not yet defined. In this study, we attempted to analyze the clinical features, to identify the major prognostic factors, and to evaluate the outcome of 121 MCL patients treated in our institution between 1979 and 1997. Clinical data, treatment modalities, and International Prognostic Index (IPI) score were evaluated. Median age was 63 years. Patients usually presented with advanced stage disease (87%), disseminated lymph nodes (57%), bone marrow involvement (79%), but with a good performance status (PS) (81%). Lymphocytosis Ͼ4000/l and/or peripheral blood involvement was present in 36% of cases, and gastrointestinal disease in 18%. The t(11;14)(q13;q32) and/or bcl-1 rearrangement was detected in 47/57 studied cases. Median overall survival (OS) was 3.12 years and a longer survival was significantly associated with younger age (Ͻ70 years), good PS (Ͻ2), localized disease (stage I-II), fewer than two extra-nodal sites, absence of spleen or peripheral blood involvement, normal serum LDH and ␤2-microglobulin levels, and hemoglobin level greater than 12 g/dl. However, the IPI failed to identify patients with longer OS and in a multiparametric analysis, only older age, hemoglobin less than 12 g/dl, poor PS, and blood involvement were associated with a poorer outcome. Treatment modalities had no impact on survival with 75% of patients relapsing or progressing. Our data showed that the poor outcome of MCL patients is mainly related to adverse patient characteristics, a highly disseminated tumor, and some unknown parameters associated with the refractoriness to standard therapy.

A Comparative Analysis of FISH, RT-PCR, PCR, and Immunohistochemistry for the Diagnosis of Mantle Cell Lymphomas

“…The differentiation of MCL from other nonHodgkin's lymphomas of peripheral B-cell type is clinically relevant because patients with MCL usually present a disseminated disease, extranodal involvement, aggressive course, and refractoriness to standard chemotherapy (5). The diagnosis of MCL first relies on morphological and phenotypical cri- teria but may be problematic when these features overlap with other B-cell lymphomas (6 -8).…”

“…It has been individualized both in the REAL and in the World Health Organization classifications because it represents a distinct clinical pathological entity with a special aggressive course among small B-cell lymphomas (2,3). It frequently involves lymph node, blood, bone marrow, and spleen (4,5). The neoplastic cells typically exhibit a centrocytic morphology and coexpress pan-B-cell markers CD5 and CD43 but lack CD23 and CD10.…”

Leukemic Mantle Cell Lymphoma: Clinical and Pathologic Spectrum of Twenty-Three Cases

“…Peripheral blood involvement has been previously reported as an independent variable of poor prognosis in patients with MCL (13,14). Previous studies also have reported that an absolute lymphocytosis of at least 10 ϫ 10 9 /L heralds shorter patient survival compared with patients with MCL and that an absolute lymphocyte count of less than 10 ϫ 10 9 /L (4).…”

Blastic mantle cell lymphoma developing concurrently in a patient with chronic myelogenous leukemia and a review of the literature

“…MCL accounts for less than 10% of all NHL and occurs in older males [18]. Patients often present with Stage III or IV disease with extension to extranodal sites including peripheral blood, bone marrow, gastrointestinal tract, and Waldeyer's ring [18][19][20].…”