B淋巴母细胞淋巴瘤的临床特点和采用改良BFM-90方案治疗的结果

The clinical features of B cell lymphoblastic lymphoma and outcomes after BFM-90 regimen therapy

目的分析 B 淋巴母细胞淋巴瘤的临床特点和采用改良 BFM-90淋巴母细胞淋巴瘤方案的治疗结果。方法对14例 B 淋巴母细胞淋巴瘤患者的临床表现进行分析,与同期住院的 T 淋巴母细胞淋巴瘤的临床表现进行比较。并对采用改良 BFM-90淋巴母细胞淋巴瘤方案治疗的疗效和不良反应进行初步分析。结果 14例 B 淋巴母细胞淋巴瘤患者,年龄3～18岁,经病理检查和免疫组化检测证实为 B 淋巴母细胞淋巴瘤。其中 I 期1例,Ⅲ期2例,Ⅳ期11例。以侵犯淋巴结和皮肤和骨髓最多见,分别占71%,50%和64%。除1例早期患者采用 CHOP+HD-MTX 化疗,其余13例均采用改良 BFM-90淋巴母细胞淋巴瘤方案化疗,其中12例患者(92.3%)获得完全缓解,1例患者(7.7%)获得部分缓解。中位随访19.5(2～44)个月,1例部分缓解患者放弃治疗后死亡,其余13例患者目前仍存活。近期疗效好,远期疗效仍须要继续随访。主要的不良反应为骨髓抑制,但均可耐受。结论 B淋巴母细胞淋巴瘤侵犯部位多为淋巴结、骨髓和皮肤,采用改良 BFM-90淋巴母细胞淋巴瘤方案治疗可改善疗效。

Objective To analyse the clinical features of patients with B cell lymphoblastie lymphoma（BCLL） and the outcomes after modified BFM-90 protocol therapy. Methods The clinical features of 14 patients with BCLL were analysed, and compared with that of T cell lymphoblastie lymphoma in the same period. The efficacy and toxicity of modified BFM-90 protocol were analysed. Results The 14 patients were aged 3 to 18 and diagnosed as BCLL by morphology and immunohistology. One case was in stage Ⅰ , 2 stage Ⅲ and 11 stage Ⅳ. Most common involved sites were lymph nodes （ 70% ） , skin （ 50% ） and bone marrow （ 64% ）. One patient received CHOP ＋ HD-MTX, and 13 recived modified BFM-90 protocol chemotherapy. Twelve patients （92.3%） achieved complete remission（ CR）, 1 patient（7.7% ） partial remission（PR）. The median follow-up duration was 19.5 months （2 to 44 months）. At present 13 patients are alive except one PR patient who gave up treatment and died of disease. The major toxicity of the protocol was myelosuppression, but could be tolerated. Conclusions The most common involvement sites of BCLL were lymph nodes, skin and bone marrow. The effectiveness is improved as treated with modified BFM-90 protocol.