| Disease | NHL of the low to intermediate grade |
| Phenotype / cell stem origin | B-cell lineage |
| Epidemiology | 5% of NHL; sex ratio: 3M/1F; median age: 65 yrs |
| Clinics | advanced disease (Ann Harbor stage III-IV) with generalized lymphadenopathies in 90%, bone marrow involvement (70%), frequent splenomegaly (50%), hepatomegaly (30%), and gastro-intestinal involvement (20%), and lymphocytosis (30%); elevated LDH in 50% |
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| Leukemic phase of mantle cell lymphoma. Peripheral blood. Small to medium sized lymphoid cells with slightly to markedly irregular nuclear contour. The nuclei have moderately dispersed chromatin bur inconspicuous nucleoli. From a patient with 46,XX,t(11;14)(q13;q32) - Courtesy Georges Flandrin. |
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| Pathology | small cleaved cell B-lymphocytes with inconspicuous nucleoli and pale cytoplasm; with a pan B-cell, CD5+, CD10-, CD23- (in contrast with CLL) phenotype; diffuse, nodular and blastoid types, the latter having large cells and higher mitotic count |
| Treatment | according to the age and the disease stage |
| Prognosis | median survival is 3 to 4 yrs; less than 2 yrs in the case of a leukaemic form (blastoid); in contrast with the good prognosis of the MALT lymphoma, a closely related disease. |
| Cytogenetics Morphological | knowledge is still scarce, and data complex t(11;14)(q13;q32) is found in 50-70% of cases, but this translocation may also, at a much lesser frequency, be found in other diseases; the genes involved in this translocation are described below; t(11;14) is found in complex karyotypes other frequent findings: deletion of parts of :1p, 6q 9p, 11q (in particular 11q22-23), 13q; gains of parts of: 3q, 8q, 15q; frequent markers; tetraploidy may be found, especially in the blastoid subtype |
| Cytogenetics Molecular | CGH (compatrative genomic hybridization) may disclose important events in this disease, taken into account that the t(11;14) may be insufficient for oncogenesis; furthermore, this and other molecular cytogenetic techniques may come in addition to the usual techniques to "dissect" complex karyotypes |
| Rearrangement and overexpression of the BCL-1/PRAD-1 gene in intermediate lymphocytic lymphomas and in t(11q13)-bearing leukemias. |
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