ABSTRACT
Intravascular large B-cell lymphoma (IVLBCL) is one of the rarest B-cell non-Hodgkin lymphomas (NHL), with an aggressive clinical behavior and a poor prognosis; in fact, its treatment is still an unmet clinical need, with a 3-year overall survival (OS) rate of 60% to 81%, and a central nervous system relapse rate of 25%. It usually presents as a widespread disease at diagnosis, with multi-organ involvement. Previously considered as a diffuse large B-cell lymphoma variant, it now represents a different extranodal large B-cell lymphoma entity in the last WHO Classification of tumors of hematopoietic and lymphoid tissues. We hereby describe the case of an 84-year-old Italian woman with an IVLBCL, cutaneous variant, who suffered from early relapse after R-COMP chemotherapy regimen, and was therefore treated with a palliative metronomic chemotherapy. Interestingly, neoplastic cells showed CD30 expression at relapse. CD30 positivity has never been reported in this disease so far, and its expression is known to be involved in NF-kB activation. CD30 expression may be further studied as for prognostic and therapeutic significance; in fact, new therapeutic strategies, such as antibody-drug conjugate targeting CD30, are now available.
Subject(s)
Gene Expression Regulation, Neoplastic , Ki-1 Antigen/biosynthesis , Lymphoma, Large B-Cell, Diffuse , Neoplasm Proteins/biosynthesis , Skin Neoplasms , Aged, 80 and over , Female , Humans , Lymphoma, Large B-Cell, Diffuse/metabolism , Lymphoma, Large B-Cell, Diffuse/pathology , Skin Neoplasms/metabolism , Skin Neoplasms/pathologyABSTRACT
Primary cutaneous acral CD8+ T-cell lymphoma (acral CD8+ TCL) is a new provisional entity characterized by acral skin lesions and an indolent course. We describe an extraordinary case characterized by relapsed nodules with CD8+ cytotoxic infiltrates on the left ear. After 35 years, the skin lesions spread to other acral sites, and a mass with the same histological features as the other skin lesions appeared on the nose. Multiple courses of chemotherapy led to stable disease. Histological examinations carried out at different times showed the gradual transformation of the neoplastic cells, with an increased proliferation index. Genomic analysis revealed losses in the regions harboring the genes involved in cell cycle control. This is the first case of an acral CD8+ TCL with a very long history of indolent nodular lesions progressing to extra-cutaneous sites.
