肝脾T细胞淋巴瘤13例临床病理分析及预后

Clinicopathological Analysis and Prognosis of 13 Cases of Hepatosplenic T-cell Lymphoma

目的探讨肝脾T细胞淋巴瘤(HSTCL)的临床病理学特点、诊断及鉴别诊断。方法收集13例HSTCL的临床资料并进行形态学观察,应用免疫组化和TCR基因重排检测,并复习相关文献。结果13例瘤细胞CD2、CD3、CD7均阳性,T细胞限制性胞内抗原TIA-1、CD56部分阳性,细胞毒性相关分子粒酶B(Granzyme-B)、EBER阴性。11例标本TCR克隆性重排检测阳性。13例患者临床治疗方案不同(脾切除术后化疗,保脾化疗,自体造血干细胞移植),随访6~58个月,预后均较差。结论HSTCL是一类罕见的结外侵袭性外周T细胞淋巴瘤,其病理表现多样且独特,结合免疫表型、TCR基因重排可确诊。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of hepatic and spleen T-cell lymphoma.Methods The clinical data of 13 cases of HSTCL were collected and observed by morphological observation.Immunohistochemistry and TCR gene rearrangement were used to examine and review related literatures.Results 13 cases of tumor cells were positive for CD2,CD3 and CD7,T cell-restricted intracellular antigens were positive for TIA-1 and CD56,and cytotoxicity-related granzyme B (Granzyme-B) and EBER were negative.11 specimens were positive for TCR clonal rearrangement.Thirteen patients had different clinical treatment regimens (chemotherapy after splenectomy,spleen-preserving chemotherapy,autologous hematopoietic stem cell transplantation),followed up for 6~58 months,and the prognosis was poor.Conclusion HSTCL is a rare type of extranodal invasive peripheral T-cell lymphoma.Its pathological manifestations are diverse and unique,and can be confirmed by immunophenotype and TCR gene rearrangement.