ABSTRACT
Objective:To analyze clinical characteristics, treatment, and prognosis of B-cell lymphoma, unclassifiable, with features in-termediate between diffuse large B-cell lymphoma and Burkitt lymphoma (DLBCL/BL). Methods:The clinical and pathological data of 13 DLBCL/BL patients, who were treated in the First Affiliated Hospital of Zhengzhou University between January 2013 and December 2014, were collected. Overall survival (OS) and progression-free survival (PFS) were estimated by the Kaplan-Meier method. Through the log-rank test, survival curves were compared among groups classified by clinical stage, age, serum lactate dehydrogenase (LDH) lev-el, international prognostic index (IPI) score, or first chemotherapy regimen. Results:Among the 13 patients with DLBCL/BL, 12 pa-tients showed extra-nodal involvement. The median OS and PFS were only 10 and 6 months, respectively. Univariate analysis showed that the LDH levels and IPI scores exerted statistically significant effects on prognosis. Some borderline differences in survival were not-ed among the CHOP, CHOP-like, and intensive chemotherapy groups. Conclusion:DLBCL/BL is an aggressive B-cell lymphoma with a short survival time. The majority of patients presented extra-nodal involvement. DLBCL/BL did not respond well to CHOP or CHOP-like regimen, and more intensive chemotherapy may improve survival. Elevated LDH levels and high IPI scores were predictors of poor sur-vival.
ABSTRACT
Gray zone lymphoma (GZL) is a special type of B-cell lymphoma,it is rare in clinical and reported fewly.In order to strengthen the understanding of GZL and make it be more recognized by clinicians.This article reviewed the status and progress of GZL by cell morphology,immunohistochemistry,cytogenetics and molecular biology,and discuss the current diagnostic criteria and treatment strategies.
ABSTRACT
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL) (intermediate DLBCL/BL), is a heterogeneous group with some features resembling DLBCL and others resembling BL. Here, we report a case of intermediate DLBCL/BL in a Korean child. A 2-yr-old male was admitted for evaluation and management of left hip pain. Immunohistochemistry of a biopsy of the femur neck revealed tumor cells positive for CD20, CD10, BCL2, BCL6, and Ki67. A bone marrow (BM) aspirate smear revealed that 49.3% of all nucleated cells were abnormal lymphoid cells, composed of large- and medium-sized cells. Immunophenotyping of the neoplastic cells revealed positivity for CD19, CD10, CD20, and sIg lambda and negativity for CD34, Tdt, and myeloperoxidase (MPO). Cytogenetic and FISH analyses showed a complex karyotype, including t(8;14)(q24.1;q32) and IGH-MYC fusion. Intensive chemotherapy was initiated, including prednisone, vincristine, L-asparaginase, daunorubicin, and central nervous system prophylaxis with intrathecal methotrexate (MTX) and cytarabine. One month after the initial diagnosis, BM examination revealed the persistent of abnormal lymphoid cells; cerebrospinal fluid cytology, including cytospin, showed atypical lymphoid cells. The patient was treated again with cyclophosphamide, vincristine, prednisone, adriamycin, MTX, and intrathecal MTX and cytarabine. The patient died of sepsis 5 months after the second round of chemotherapy.
Subject(s)
Child, Preschool , Humans , Male , Antineoplastic Agents/therapeutic use , Bone Marrow Cells/pathology , Chromosomes, Human, Pair 14 , Chromosomes, Human, Pair 8 , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Drug Therapy, Combination , Femur Neck/pathology , Immunohistochemistry , Immunophenotyping , In Situ Hybridization, Fluorescence , Karyotyping , Lymphoma, B-Cell/diagnosis , Methotrexate/therapeutic use , Oncogene Proteins, Fusion/genetics , Prednisolone/therapeutic use , Republic of Korea , Translocation, Genetic , Treatment Outcome , Vincristine/therapeutic useABSTRACT
As a distinct clinicopathological entity, mediastinal gray zone lymphoma cannot be classified by using differential diagnostic criteria. The cases represent a spectrum of tumors having characteristics of both primary mediastinal large B-cell lymphoma (PMBL) and classic Hodgkin lymphoma (cHL). In the 2008 WHO Classification, a novel category designated B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma (BCLu) has been created to include these neoplasms. The main features of BCLu are clearly different from those of conventional mediastinal large B-cell lymphomas. Diagnosis of BCLu requires a multiparameter approach incorporating morphological, immunophenotypic, immunohistochemical and other features. These lymphomas generally have a more aggressive clinical course and poorer outcome. There is no consensus on the optimum treatment, while the recommended therapies for aggressive B-cell lymphomas might be effective options.