tailieunhanh - Ebook The cutaneous lymphoid proliferations - A comprehensive textbook of lymphocytic infiltrates of the skin (2nd edition): Part 2

(BQ) Part 1 book "The cutaneous lymphoid proliferations - A comprehensive textbook of lymphocytic infiltrates of the skin" presents the following contents: Cutaneous mantle cell lymphoma, mycosis fungoides and sézary syndrome, subcutaneous panniculitis like t cell lymphoma, nasal and related extranodal natural killer cell T cell lymphomas and blastic plasmacytoid dendritic cell neoplasm,. | CHAPTER 11 Cutaneous Mantle Cell Lymphoma Clinical features Mantle cell lymphoma is a mature B cell lymphoma manifesting a characteristic morphology phenotype and molecular profile it is an aggressive form of B cell lymphoma accounting for 3-10 of all cases of non-Hodgkin lymphoma Banks et al. 1992 Campo et al. 1999 see Table . Weisenburger and coworkers first recognized the entity using the designation of diffuse intermediate lymphocytic lymphoma Weisenburger et al. 1981 . Mantle cell lymphoma is associated with a poor prognosis and has remained largely incurable with current chemotherapeutic approaches although patients often have an initial response to chemotherapy most develop progressive disease and succumb to it. Mantle cell lymphoma Clinical Adults Most often multiple tumors Typically in the context of nodal disease and hence represents Stage IV disease rare cases of primary cutaneous mantle cell lymphoma Histomorphology Monomorphous small- to medium-sized lymphocytic proliferation Characteristically in the skin a blastic morphology is seen Immunophenotype CD20 CD5 rare cases are CD5- Cyclin-D1 bcl-1 occasionally negative including blastoid areas CD23CD10CD8 positivity rarely Genetics t 11 14 q13 q32 del 11 q . del 13 q Therapy Systemic chemotherapy Bone marrow transplantation The disease primarily affects Caucasians and is uncommon in patients of African descent. The mean age at presentation is 60 years and there is a male predominance of 4 1. Disease is often widespread at initial presentation with lymph node spleen liver and or bone marrow involvement. Peripheral blood lymphocytosis is found in at least one-quarter of cases. When progressive dissemination occurs the gastrointestinal tract and Waldeyer s ring are preferential sites of involvement. Most cases of intestinal lymphomatosis represent mantle cell lymphoma the gross morphology is characterized by multiple sized polyps affecting any segment of the gastrointestinal tract .