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目的:探讨青少年急性淋巴细胞白血病(ALL)孤立卵巢复发的临床病理特点及诊断和治疗。方法:1例16岁青少年女性以骨关节疼痛起病,外周血涂片分类见原幼淋巴细胞32%。骨髓细胞学检查示增生明显活跃,原幼淋巴细胞72%。骨髓细胞流式细胞免疫分型显示,原始细胞表达HLA-DR、Td T、CD10、CD19、CD22和c CD79a。骨髓细胞染色体和融合基因检查显示,t(1;19)(q23;p13)/E2A-PBX1阳性,确诊B-ALL。采用CALGB8811方案治疗,完全缓解7个月后出现下腹痛,超声检查诊为卵巢肿瘤。行双侧卵巢肿块切除术。结果:卵巢病理检查发现弥漫性幼稚淋巴细胞浸润,免疫组织化学检测显示瘤细胞表达Td T、CD20、CD43和CD79a,证实为ALL卵巢复发。进一步检查未发现其它髓外部位病变。同期的血液和骨髓细胞学检查仍显示完全缓解,但流式细胞免疫分型检测微小残留白血病阳性。再次诱导化疗后病情控制,1年后肿块再次增大,并压迫输尿管致肾积水,后患者放弃治疗。结论:尽管孤立性卵巢复发在ALL极为少见,但预后极差,对骨髓或其它髓外部位复发的女性患者,应进行常规检查以排除此种可能。
Objective: To investigate the clinicopathological features, diagnosis and treatment of isolated ovarian relapse in adolescent acute lymphoblastic leukemia (ALL). Methods: A 16-year-old adolescent female with bone-joint pain onset, peripheral blood smear classification see the original immature lymphocytes 32%. Bone marrow cytology showed significantly hyperplasia, young lymphocytes 72%. Flow cytometric immunoblotting of bone marrow cells showed that the primitive cells expressed HLA-DR, Td T, CD10, CD19, CD22 and CD79a. Bone marrow cell chromosome and fusion gene tests showed that t (1; 19) (q23; p13) / E2A-PBX1 was positive and B-ALL was confirmed. Treatment with CALGB8811 regimen, complete remission of abdominal pain after 7 months, ultrasound diagnosis of ovarian cancer. Line bilateral ovarian tumor resection. Results: Diffuse immature lymphocytes infiltrated by ovarian pathological examination. Immunohistochemistry showed that tumor cells expressed Td T, CD20, CD43 and CD79a, which was confirmed as ALL ovarian recurrence. Further examination found no other extramedullary lesions. The same period of blood and bone marrow cytology still showed complete remission, but the flow cytometry positive detection of minimal residual leukemia. Chemotherapy after induction of disease control, 1 year after the tumor increases again, and urinary hydronephrosis, after giving up treatment. CONCLUSIONS: Although isolated ovarian recurrence is rare in ALL, the prognosis is poor, and routine testing should be performed to exclude this possibility in women with bone marrow or other extramedullary site recurrences.