Objective To investigate the clinical features, pathological characteristics, treatment and prognosis of primary cutaneous pre B-cell lymphoblastic lymphoma (B-LBL) in children, and raise awareness of this disease as well. Methods Two cases of primary cutaneous B-LBL were reported and the related literatures were reviewed. Results The onset of symptoms were skin lesions on the left knee and right jaw with local lymphadenopathy. It was misdiagnosed as cutaneous infection at the beginning, and showed ineffective in anti-infection therapy. After pathological biopsies, they were diagnosed as primary cutaneous B-LBL. Besides, bone marrow was also involved. Therefore, they were classified into stage Ⅳ. After admission, they were treated with BCH-LBL-2003 chemotherapy regimen, and remained in complete remission for 26 and 31 months from diagnosis. Conclusions Only cutaneous involvement was detected at the beginning of this disease in these two patients, so it is easy to miss diagnosis or make misdiagnosis. Pathological biopsy should be performed as soon as possible. Bone marrow biopsy is also needed for clinical stratification. Combination chemotherapy of multi-drugs leads to favorable prognosis.%目的 了解儿童原发性皮肤前B淋巴母细胞性淋巴瘤的临床、病理特点以及治疗和预后,提高对本病的认识.方法 对2例患儿资料进行临床病例分析及文献复习.结果 2例患儿分别以左膝关节及右颌下皮肤皮疹起病,均伴有局部淋巴结肿大,早期被误诊为皮肤感染,抗感染治疗无效,经皮肤活检病理确诊为前B淋巴母细胞性淋巴瘤,骨髓活检病理提示骨髓受累,就诊时临床分期即为Ⅳ期,按BCH-LBL-2003方案规律化疗,分别随诊26及31个月,至今完全缓解无复发.结论 该病早期仅有皮肤受累,易误诊或漏诊,怀疑本病应尽早做活检病理检查确诊,并进行全面评估分期,骨髓活检对该病分期诊断具有重要意义,多药联合化疗预后较好.
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