Large B-Cell Lymphoma Initially Presenting in Bone Marrow, Liver and Spleen: An Aggressive Entity Frequently Associated with Hemophagocytic Syndrome
KC Chang, CC Chen, IC Liao, SY Tsai, DM Jones. National Cheng Kung University, Tainan, Taiwan; The University of Texas M. D. Anderson Cancer Center, Houston
Background: Diffuse large B-cell lymphoma (DLBCL), the most common type of malignant lymphoma, contains several distinctive variants in the WHO classification scheme. Recently, several cases manifesting initially in bone marrow, liver and spleen (BLS-type) without lymphadenopathy have been reported.
Design: We describe the clinicopathologic and cytogenetic features of 11 such cases (8 men, 3 women; mean age: 62.7 years old) from two centers in Taiwan and the United States.
Results: Usually presenting with fever and hemophagocytic syndrome suggesting infection and complicating timely diagnosis, bone marrow examination showed patchy and interstitial infiltration of large tumor cells without sinusoidal involvement. All cases had a high proliferative rate (≥ 90% Ki-67+), commonly a non-germinal center/activated B-cell immunophenotype and were negative for Epstein-Barr virus by in situ hybridization. The most frequent cytogenetic changes involved chromosomal loci 14q32 and 9p24, as well as del(3)(q21), add(7)(p22), t(3;6), del(8)(p22), +18, and add(19)(p13). The clinical behavior was very aggressive with a 2-year survival rate of 18% (45% of patients died within 3 weeks). High-dose chemotherapy with hematopoietic stem cell transplantation prolonged survival in several cases.
Conclusions: Although it shares with intravascular LBCL a subtle presentation and an aggressive clinical course, this primary BLS large cell lymphoma variant is distinguished by lacking an intravascular component and having different cytogenetic findings.
Wednesday, March 24, 2010 9:30 AM
Poster Session V # 181, Wednesday Morning