Abstract
BackgroundDiffuse large B-cell lymphoma (DLBCL) of leukemic phase is a rare clinical manifestation, but is highly prevalent with central nervous system involvement (CNSI). Little is known about this rare clinical observation.MethodsWe reviewed the clinical characteristics of 40 DLBCL patients with leukemic phase identified by flow cytometry and analyzed BCL2 and MYC aberrations by fluorescence in situ hybridization.ResultsThe median age of these 40 patients was 46 years (range, 15–75) with 19 men patients. All patients had bone marrow involvement, and fourteen (35.0%) had CNSI. There were respectively 14 patients (35.0%) had the BCL2 or MYC gain/amplification and nine of them (22.5%) simultaneously had both aberrations. Compared to those without CNSI, CNSI was found more commonly in male patients (71.4 vs. 34.6%, p = 0.046), in those with IPI scores of 4–5 (57.1% vs. 11.5%, p = 0.001), and in those with elevated serum LDH (100 vs. 61.5%, p = 0.007) and both MYC and BCL2 rearrangement (88.9 vs. 19.4%; p = 0.000). BCL2 and MYC rearrangements were the sole independent factor correlated with CNSI.ConclusionIt is possible that both BCL2 and MYC gene aberrations may contribute to the high incidence of CNSI observed in leukemic phase of patients with DLBCL.
Highlights
Diffuse large B-cell lymphoma (DLBCL) of leukemic phase is a rare clinical manifestation, but is highly prevalent with central nervous system involvement (CNSI)
Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid neoplasm, which account for 30%-40% of non-Hodgkin lymphoma (NHL) [1] in the Western countries and more than 50% of NHL in China
We demonstrated that concomitant BCL2 and MYC abnormalities were the only independent factor that correlated with CNS involvement
Summary
Diffuse large B-cell lymphoma (DLBCL) of leukemic phase is a rare clinical manifestation, but is highly prevalent with central nervous system involvement (CNSI). Little is known about this rare clinical observation. Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid neoplasm, which account for 30%-40% of non-Hodgkin lymphoma (NHL) [1] in the Western countries and more than 50% of NHL in China. DLBCL is a heterogeneous group of disorders with variable histological and clinical behavior. Up to 40% of patients may have extranodal involvement at diagnosis. The common extranodal sites include the gastrointestinal tract, bone, bone marrow, testis, salivary gland, adrenal gland, liver, Zou et al BMC Medical Genetics (2017) 18:16 amplification were strong independent indicators of CNS involvement in DLBCL patients at leukemic phase
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