Diffuse large B-cell lymphoma with concomitant c-MYC and BCL6 gene rearrangements with primary skin involvement: A case report and a review of literature

• Published in: Terapevtic̆eskii arhiv Vol. 89; no. 7; pp. 85 - 92
• Main Authors: Gabeeva, N G, Koroleva, D A, Belyaeva, A V, Chernova, N G, Kuzmina, L A, Sudarikov, A B, Obukhova, T N, Kovrigina, A M, Zvonkov, E E, Savchenko, V G
• Format: Journal Article
• Language: English; Russian
• Published: Russia (Federation) "Consilium Medicum" Publishing house 01-01-2017
• Subjects: Antineoplastic Agents - administration & dosage; Antineoplastic Agents - adverse effects; Antineoplastic Combined Chemotherapy Protocols - pharmacology; bcl2; bcl6; dhl; dlbcl leg-type; double-hit lymphoma (dhl); Female; Gene Rearrangement; Genes, myc - genetics; Humans; Lung Neoplasms - pathology; Lung Neoplasms - secondary; Lymphoma, Large B-Cell, Diffuse - genetics; Lymphoma, Large B-Cell, Diffuse - pathology; Lymphoma, Large B-Cell, Diffuse - physiopathology; Lymphoma, Large B-Cell, Diffuse - therapy; Male; Middle Aged; Neoplasm Staging; Proto-Oncogene Proteins c-bcl-6 - genetics; Pyrazoles - administration & dosage; Pyrimidines - administration & dosage; Skin - pathology; Skin Neoplasms - genetics; Skin Neoplasms - pathology; Skin Neoplasms - physiopathology; Skin Neoplasms - therapy; Treatment Outcome; BCL2; double-hit lymphoma (DHL); BCL6; DLBCL leg-type; с-MYC
• ISSN: 0040-3660; 2309-5342
• DOI: 10.17116/terarkh201789785-92

Double-hit lymphoma (DHL) is a rare aggressive B-cell lymphoma with concomitant c-MYC, BCL2 or BCL6 gene rearrangements, which is characterized by the high frequency of extranodal lesions and by resistance to chemotherapy. The median survival does not exceed 18 months in patients with this disease. The majority of DHL is represented by с-MYC/BCL2 cases. The combination of c-MYC/BCL6 occurs rarely (5-8%). The paper describes a case of DHL with concomitant c-MYC and BCL6 gene rearrangements, which mimics diffuse large B-cell lymphoma, leg-type.