TY - JOUR
T1 - Double hit lymphoma
T2 - The MD Anderson Cancer Center clinical experience
AU - Oki, Yasuhiro
AU - Noorani, Mansoor
AU - Lin, Pei
AU - Davis, Richard E.
AU - Neelapu, Sattva S.
AU - Ma, Long
AU - Ahmed, Mohamed
AU - Rodriguez, Maria Alma
AU - Hagemeister, Fredrick B.
AU - Fowler, Nathan
AU - Wang, Michael
AU - Fanale, Michelle A.
AU - Nastoupil, Loretta
AU - Samaniego, Felipe
AU - Lee, Hun J.
AU - Dabaja, Bouthaina S.
AU - Pinnix, Chelsea C.
AU - Medeiros, Leonard J.
AU - Nieto, Yago
AU - Khouri, Issa
AU - Kwak, Larry W.
AU - Turturro, Francesco
AU - Romaguera, Jorge E.
AU - Fayad, Luis E.
AU - Westin, Jason R.
PY - 2014/9
Y1 - 2014/9
N2 - We report our experience with 129 cases of double hit lymphoma (DHL), defined as B-cell lymphoma with translocations and/or extra signals involving MYC plus BCL2 and/or BCL6. All cases were reviewed for histopathological classification. Median age was 62 years (range, 18-85), 84% of patients had advanced-stage disease, and 87% had an International Prognostic Index score ≥2. Fourteen patients (11%) had a history of low-grade follicular lymphoma. MYC translocation was present in 81%, and extra signals of MYC in 25% of patients. IGH-BCL2 translocation was present in 84% and extra signals of BCL2 in 12% of patients. Two-year event-free survival (EFS) rates in all patients and patients who received R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone), R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin), and R-HyperCVAD/MA (rituximab, hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone, alternating with cytarabine plus methotrexate) were 33%, 25%, 67% and 32%, respectively. In patients achieving complete response with initial therapy (n = 71), 2-year EFS rates in patients who did (n = 23) or did not (n = 48) receive frontline stem cell transplantation were 68% and 53%, respectively (P = 0·155). The cumulative incidence of central nervous system involvement was 13% at 3 years. Multivariate analysis identified performance status ≥2 and bone marrow involvement as independent adverse prognostic factors for EFS and OS. Further research is needed to identify predictive and/or targetable biological markers and novel therapeutic approaches for DHL patients.
AB - We report our experience with 129 cases of double hit lymphoma (DHL), defined as B-cell lymphoma with translocations and/or extra signals involving MYC plus BCL2 and/or BCL6. All cases were reviewed for histopathological classification. Median age was 62 years (range, 18-85), 84% of patients had advanced-stage disease, and 87% had an International Prognostic Index score ≥2. Fourteen patients (11%) had a history of low-grade follicular lymphoma. MYC translocation was present in 81%, and extra signals of MYC in 25% of patients. IGH-BCL2 translocation was present in 84% and extra signals of BCL2 in 12% of patients. Two-year event-free survival (EFS) rates in all patients and patients who received R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone), R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin), and R-HyperCVAD/MA (rituximab, hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone, alternating with cytarabine plus methotrexate) were 33%, 25%, 67% and 32%, respectively. In patients achieving complete response with initial therapy (n = 71), 2-year EFS rates in patients who did (n = 23) or did not (n = 48) receive frontline stem cell transplantation were 68% and 53%, respectively (P = 0·155). The cumulative incidence of central nervous system involvement was 13% at 3 years. Multivariate analysis identified performance status ≥2 and bone marrow involvement as independent adverse prognostic factors for EFS and OS. Further research is needed to identify predictive and/or targetable biological markers and novel therapeutic approaches for DHL patients.
KW - BCL2
KW - BCL6
KW - Double hit lymphoma
KW - MYC
KW - Prognostic factors
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U2 - 10.1111/bjh.12982
DO - 10.1111/bjh.12982
M3 - Article
C2 - 24943107
AN - SCOPUS:84907599085
SN - 0007-1048
VL - 166
SP - 891
EP - 901
JO - British Journal of Haematology
JF - British Journal of Haematology
IS - 6
ER -