PDF(Pubmed)
Abstract：
Lymphoblastic lymphoma is a rare and aggressive form of non-Hodgkin lymphoma (NHL). The tumor can derive from T-cell or B-cell and is clinically similar to acute lymphoblastic leukemia with minimal to no bone marrow involvement distinguishing the two. We present a rare case of lymphoblastic lymphoma originating from the paranasal sinuses. A 40-year-old male presented to the emergency department and was diagnosed with right-sided acute sinusitis complicated by pre-septal cellulitis. After failing medical management, he underwent endoscopic sinus surgery. Pathologic analysis of nasal contents stained for CD79a, CD34, and PAX5, suggesting a B-cell lymphoblastic lymphoma (B-LBL). He was referred to hematology-oncology and was treated with hyperfractionated cyclophosphamide, vincristine, Adriamycin, dexamethasone (Hyper-CVAD). Short-term follow-up has thus far demonstrated near-complete resolution of the tumor. Non-Hodgkin lymphomas of the paranasal sinuses are rare, and B-cell lymphoblastic lymphomas comprise just 0.3% of adults with NHL. Immunohistochemical phenotyping for B-LBL is typically positive for B-cell markers CD19, CD20, CD22 and CD79a. Classic treatment involves a chemotherapy regimen of Hyper-CVAD with an overall survival rate of 66%. B-cell lymphoblastic lymphoma is rarely reported in the paranasal sinuses. A thorough history and physical exam along with a complete workup, including biopsies with histopathological and immunohistochemical analysis, needs to be obtained. Little is known about its prognosis when the primary site is within the paranasal sinuses, and therefore, patients need prompt and aggressive treatment when the diagnosis is made.
摘要：
淋巴母细胞淋巴瘤是一种罕见且侵袭性的非霍奇金淋巴瘤（NHL）。肿瘤可以来自T细胞或B细胞，在临床上与急性淋巴细胞白血病相似，仅有很小的骨髓参与，甚至没有骨髓参与。我们介绍了一例罕见的源自鼻旁窦的淋巴母细胞淋巴瘤。一名40岁的男性出现在急诊科，被诊断为右侧急性鼻窦炎并伴有鼻中隔前蜂窝织炎。在医疗管理失败后，他接受了内窥镜鼻窦手术。鼻腔内容物CD79a染色病理分析,CD34和PAX5，提示B细胞淋巴母细胞淋巴瘤（B-LBL）。他被转诊到血液肿瘤学，并接受超分割环磷酰胺治疗，长春新碱,阿霉素,地塞米松（Hyper-CVAD）。到目前为止，短期随访已证明肿瘤几乎完全消退。鼻旁窦的非霍奇金淋巴瘤很少见，B细胞淋巴母细胞淋巴瘤仅占成人NHL的0.3％。B-LBL的免疫组织化学表型通常对B细胞标志物CD19、CD20、CD22和CD79a呈阳性。经典治疗包括Hyper-CVAD的化疗方案，总生存率为66％。B细胞淋巴母细胞淋巴瘤在鼻旁窦很少报道。全面的历史和身体检查以及完整的检查，包括组织病理学和免疫组织化学分析活检，需要获得。当主要部位在鼻旁窦内时，对其预后知之甚少。因此,患者在诊断时需要及时积极的治疗.
