International Journal of Clinical and Experimental Pathology
Int J Clin Exp Pathol 1(1):65-74;2008
Original Article C-MYC Rearrangements are Frequent in Aggressive Mature B-Cell Lymphoma with Atypical Morphology
Xianfeng F. Zhao, Anjum Hassan, Arie Perry, Yi Ning, Sanford A. Stass and Louis P. Dehner
Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO and Department of Pathology, University of Maryland School of Medicine, Baltimore, MD
Received 24 May 2007; accepted 18 June 2007; available online 1 January 2008
Abstract: Diagnosing aggressive mature B-cell lymphomas with atypical morphology remains a potential challenge. Although most of these lymphomas were diagnosed as diffuse large B-cell lymphoma (DLBCL), more and more molecular studies indicate that overlap exists between Burkitt lymphoma (BL) and DLBCL, raising important concerns in making the final diagnosis. To identify possible factors that could contribute to the atypical morphology of these lymphomas, we selected eight challenging cases of aggressive mature B-cell lymphomas with atypical morphology and evaluated them using morphologic, immunophenotypic and cytogenetic data with clinical correlations. Morphologically, the neoplastic cells showed a diffuse monotonous infiltrating pattern. The neoplastic cells displayed a spectrum of morphologic features that we classified into four categories based on existing morphologic standards: 1) L1 lymphoblastic; 2) centroblastic; 3) immunoblastic; and 4) mixed centroblastic and immunoblastic. Most of these cases were positive for CD10 and/or BCL6, and show BCL2 expression. However, c-MYC rearrangements were demonstrated in 75% of the cases (6/8). Interestingly, the c- MYC rearrangements were detected in six cases with labeling indices <90%, whereas c-MYC rearrangements were not identified in two others with >95% labeling indices. A t(14;18) was additionally detected in three cases with c-MYC rearrangements. Clinical follow up indicates that some of these aggressive mature B-cell lymphomas with c-MYC rearrangements may have benefited from more intensified chemotherapy like that used for Burkitt lymphoma. Our study suggests that the aggressive mature B-cell lymphomas with atypical features may be another “grey zone lymphoma” lying in the spectrum between BL and DLBCL. (IJCEP705003).
Key Words: atypical mature B-cell lymphoma; Burkitt lymphoma; diffuse large B-cell lymphoma; grey zone lymphoma; c-MYC rearrangement
Address all correspondences to: XianFeng F. Zhao, MD, PhD, Hematopathology Section, Department of Pathology, University of Maryland School of Medicine, 22 S Greene Street, NBW78, Baltimore, MD 21201, USA. Tel: 410-328-5555; Fax:410-328-5508; Email: xzhao@umm.edu.