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CLINICAL FEATURES IN DLBCL AND TRANSLOCATION BCL2/c-MYC “DOUBLE HIT” LYMPHOMA
ŽELJKA ŠKUNCA
; Zadar General Hospital, Department of Hematology, Zadar, Croatia
MARA DOMINIS
; Merkur University Hospital, Department of Clinical Pathology, Zagreb, Croatia
ANA PLANINC-PERAICA
; Merkur University Hospital, Department of Hematology, Zagreb, Croatia
BRANIMIR JAKŠIĆ
; Klinička bolnica Merkur, Klinika za unutarnje bolesti, Zagreb, Hrvatska
Abstract
Diffuse large B-cell lymphoma (DLBCL) is classified as lymphoma and various entities using the gene expression of proteins are classified into three groups. The aim of this study was to clarify the clinical, biological, immunophenotypic and cytogenetic features of DLBCL with translocation t (14; 18) and 8q24/c-MYC. Eleven DLBCL patients with dual translation were monitored during the 2000-2009 period. The characteristics of these patients included morphological, immunohistochemical and cytogenetic analysis. Study results showed that all patients had aggressive characteristics, presence of B symptoms (64%), general patient condition according to ECOG scale ≥2 (55%), elevated serum lactate dehydrogenase activity (73%), clinical stage III and IV (82%), extranodal involvement of the disease (73%), and IPI ≥2 (73%). Partial remission was achieved in 73% of all patients and all patients (73%) died within a short time. Patients were treated with CHOP and similar protocols (COP, CVP, CNOP), with the addition of MabThera. Immunophenotyping was performed and determined expression of the CD20, CD3, CD10, BCL6 and MUM1 markers. The cytogenetic analysis/fluorescence in situ hybridization revealed complex karyotype changes. Thus, we analyzed the presence of BCL2, BCL6 and c-MYC genes and found eight patients to have BCL2 and c-MYC translocation genes, while three had translocation of the BCL6 and c-MYC genes. Despite appropriate therapy, the patient prognosis is poor. The median survival in these patients was 1.85 years. DLBCL with BCL2 and c-MYC rearrangement of the subgroups of lymphoma is associated with very poor survival. The presence of these two translocations has an aggressive clinical course.
Keywords
diffuse large B-cell lymphoma; BCL2; c-MYC
Hrčak ID:
136991
URI
Publication date:
27.3.2015.
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