Smyth L, Browne P V, Conneally E, Flynn C, Hayden P, Jeffers M, O'Brien D, Quinn F, Kelly J, Perera M, Crotty G M, Leahy M, Hennessy B, Jackson F, Ryan M, Vandenberghe E
Department of Haematology, St James's Hospital, Dublin 8, Ireland.
Department of Histopathology, AMNCH, Dublin 24, Ireland.
Ir J Med Sci. 2016 Nov;185(4):773-777. doi: 10.1007/s11845-015-1288-3. Epub 2015 Apr 7.
Sporadic Burkitt lymphoma (BL), characterised by translocation-associated C-MYC upregulation is a rare, aggressive lymphoma with a cure rate up to 90 % using the R-CODOX-M/R-IVAC (RCRI) protocol. RCRI is active in HIV-associated BL in combination with HAART. The WHO classification system defines lymphomas intermediate between DLBCL and BL, in which lymphomas with t(14;18)(q32;q21) and C-MYC-carrying translocation, i.e. 'double-hit' are included (BL-DH), and these patients are conventionally treated with RCRI.
We describe the SJH experience of 25 patients with BL, BL + HIV and BL-DH treated with RCRI between 2002 and 2011. Twelve BL patients (8M/4F), median age 49.1 years (range 20-73 years); of whom 9 had extensive disease, including 8 with marrow and 2 with CNS involvement. Eleven patients remain in remission at 80.5 months (range 37-147 months) from completion of treatment and one died of progressive BL giving an OS of 91.6 % at 1 year with no late relapses. Eight patients with BL + HIV were treated (6M/2F) with a median age 40.25 years (range 24-64). Five remain in complete remission (CR) at 65 months (range 13-109 months), three patients died, two of progressive disease and one of treatment-associated hepatotoxicity in CR. Five patients with BL-DH were included; (3M/2F), age 47.8 years (range 42-55 years); and all patients died of progressive disease, 4 on RCRI therapy and a further patient despite an allogeneic transplantation.
These results confirm that RCRI is an effective treatment in adults with BL and BL + HIV and remains the gold standard against which other regimens should be compared. We confirm the poor prognosis found in BL-DH, indicating new treatment approaches are needed for this sub-group which should be identified at diagnosis by FISH analysis.
散发性伯基特淋巴瘤（BL）以与易位相关的C-MYC上调为特征，是一种罕见的侵袭性淋巴瘤，使用R-CODOX-M/R-IVAC（RCRI）方案治愈率高达90%。RCRI与高效抗逆转录病毒治疗（HAART）联合应用于HIV相关BL时具有活性。世界卫生组织分类系统定义了介于弥漫性大B细胞淋巴瘤（DLBCL）和BL之间的淋巴瘤，其中包括具有t(14;18)(q32;q21)和携带C-MYC易位的淋巴瘤，即“双打击”淋巴瘤（BL-DH），这些患者传统上采用RCRI治疗。
我们描述了2002年至2011年间在SJH接受RCRI治疗的25例BL、BL+HIV和BL-DH患者的情况。12例BL患者（8例男性/4例女性），中位年龄49.1岁（范围20-73岁）；其中9例有广泛病变，包括8例骨髓受累和2例中枢神经系统受累。11例患者在完成治疗后80.5个月（范围37-147个月）仍处于缓解期，1例死于进行性BL，1年总生存率为91.6%，无晚期复发。8例BL+HIV患者（6例男性/2例女性）接受治疗，中位年龄40.25岁（范围24-64岁）。5例在65个月（范围13-109个月）时仍处于完全缓解（CR）状态，3例患者死亡，2例死于进行性疾病，1例在CR期死于治疗相关的肝毒性。纳入5例BL-DH患者；（3例男性/2例女性），年龄47.8岁（范围42-55岁）；所有患者均死于进行性疾病，4例在RCRI治疗期间死亡，另1例尽管接受了异基因移植仍死亡。
这些结果证实RCRI是成人BL和BL+HIV的有效治疗方法，仍然是应与其他方案进行比较的金标准。我们证实了BL-DH预后较差，表明该亚组需要新的治疗方法，应在诊断时通过荧光原位杂交（FISH）分析进行识别。
