Prognostic value of MYC rearrangement in cases of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma

Lin, Pei ; Dickason, Timothy J. ; Fayad, Luis E. ; Lennon, Patrick A. ; Hu, Peter ; García, Mar ; Routbort, Mark J. ; Miranda, Roberto ; Wang, Xumei ; Qiao, Wei ; Medeiros, L. Jeffrey

Resumen: BACKGROUND: B-cell lymphoma, Unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, for convenience referred to here as unclassifiable B-cell lymphoma, is a category in the 2008 World Health Organization system used for a group of histologically aggressive neoplasms that are difficult to classify definitively. Currently, there is no established standard therapy for these neoplasms.
METHODS: The authors assessed MYC status and correlated it with treatment response and outcome in a group of 52 patients with unclassifiable B-cell lymphoma treated with either a standard DLBCL regimen (R-CHOP [rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone-related therapy]) or more intensive regimens, such as R-hyper-CVAD (rituximab plus hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with high-dose methotrexate and cytarabine). The regimens were selected by the treating clinicians based on the overall clinical and pathological findings.
RESULTS: Thirty (58%) unclassifiable B-cell lymphomas had MYC abnormalities (MYC+) including 27 with rearrangement, 2 with amplification, and 1 with both. The MYC+ and MYC− groups were similar in their age distribution and International Prognostic Index scores. Progression-free survival of patients with MYC+ unclassifiable B-cell lymphoma treated initially with R-CHOP was significantly worse than patients treated with R-hyper-CVAD (P = .0358). In contrast, for the MYC− unclassifiable B-cell lymphoma group, some patients responded to R-CHOP, and others were refractory to R-hyper-CVAD.
CONCLUSIONS: MYC aberrations are common in unclassifiable B-cell lymphoma. The presence of MYC aberrations identifies a patient subset that requires more aggressive therapy than R-CHOP. In contrast, MYC− unclassifiable B-cell lymphoma patients responded variably to either R-CHOP or aggressive therapy, and the latter showed no survival advantage.

Idioma: Inglés

DOI: 10.1002/cncr.26433

Año: 2012

Publicado en: CANCER 118, 6 (2012), 1566-1573
ISSN: 0008-543X

Factor impacto JCR: 5.201 (2012)

Categ. JCR: ONCOLOGY rank: 32 / 197 = 0.162 (2012) - Q1 - T1

Tipo y forma: Article (Published version)