Objective: To study the clinicopathologic features, diagnosis and differential diagnosis of tumors of haematopoietic and lymphoid tissue in the female productive tract. Methods: Eleven cases of myeloid sarcoma and leukemia, 9 of non Hodgkin lymphoma (NHL) , 13 of cervical lymphoma-like lesions were selected from Peking University People's Hospital from January 2006 to August 2017. According to WHO classification of tumors of haematopoietic and lymphoid tissues (2008) and updated classification(2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization. Results: In 20 cases of tumors of haematopoietic and lymphoid tissue, the mean and median age was 48.5 and 56 years old (range: 16-77 years old) . In cases of lymphoma-like lesion of uterine cervix, the mean and median age was 45.9 and 48 years old (range: 23-62 years old) . The patients with neoplasm present as fever, fatigue, hypogastralgia, colporrhagia and mass etc. Eight cases had history of acute myeloid leukemia, and 3 had myeloid leukemia while pregnancy. One case of chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL) had history of ovary small cell carcinoma and high grade serous carcinoma resected with chemotherapy. One case of diffuse large B cell lymphoma (DLBCL) had history of renal transplantation. Lactic dehydrogenase (LDH) was elevated in 9 cases (9/18) . The cases of lymphoma-like lesion present as contact bleeding in most cases and all located in cervix. Four cases of neoplasm located in vulva, 1 in vagina, 4 in cervix, 4 in uterine corpus, 8 in ovary and 2 in placenta. Clinical staging of NHL: 4 case was stageⅠ, 1 case of stageⅢ, and 4 cases of stage Ⅳ. Pathological morphology: 9 cases were myeloid sarcoma, 2 cases were placenta invaded by myeloid leukemia. Six cases were DLBCL, and 1 case was CLL/SLL, 1 case was mucosa associated lymphoid tissuse lymphoma (MALToma) , and 1 case was anaplastic large cell lymphoma. Resected mass, chemotherapy was performed in tumors of haematopoietic and lymphoid tissue. Five cases of myeloid sarcoma and 2 of NHL died. In 13 cases of lymphoma-like lesion of uterine cervix, the general condition was good as following up. Conclusions: The clinical history, pathological morphology and immunohistochemistry are very important for diagnosing tumors of haematopoietic and lymphoid tissue in the female productive tract. Resection with chemotherapy is recommended in treatment. The prognosis of lymphoma-like lesion of uterine cervix is good, and should be differentiated from lymphoma.
目的： 探讨生殖系统的淋巴造血组织增生性病变（包括淋巴造血组织肿瘤和淋巴瘤样病变）的临床病理特征。 方法： 收集2006年1月至2017年8月北京大学人民医院收治的生殖系统出现首发症状的淋巴造血组织增生性病变患者共33例。其中，生殖系统的淋巴造血组织肿瘤20例，包括髓细胞肉瘤和髓性白血病11例、原发性生殖系统非霍奇金淋巴瘤（NHL）9例；子宫颈淋巴瘤样病变13例。应用光镜观察、免疫组化染色、EB病毒原位杂交及临床相关检查对其临床病理特征进行回顾性分析。 结果： （1）年龄：20例淋巴造血组织肿瘤患者的年龄范围为16~77岁，平均年龄48.5岁，中位年龄56岁。13例子宫颈淋巴瘤样病变患者的年龄范围为23~62岁，平均年龄45.9岁，中位年龄48岁。（2）临床表现：20例生殖系统的淋巴造血组织肿瘤患者的临床表现为发热、乏力、下腹痛、阴道流血，以及肿物等；13例子宫颈淋巴瘤样病变患者的临床表现多为接触性出血。（3）病史：20例淋巴造血组织肿瘤患者中，8例患者有急性髓性白血病病史，3例患者妊娠合并髓性白血病病史；1例慢性淋巴细胞白血病/小细胞性淋巴瘤（CLL/SLL）患者有卵巢小细胞癌和高级别卵巢浆液性癌术后化疗病史，1例弥漫性大B细胞淋巴瘤（DLBCL）患者有肾移植术后应用环孢素病史。（4）实验室检查：18例有血清乳酸脱氢酶（LDH）水平检测结果的淋巴造血组织肿瘤患者中，9例（9/18）血清LDH水平升高。（5）肿瘤部位：20例淋巴造血组织肿瘤患者中，肿瘤位于外阴4例，阴道1例，子宫颈4例，子宫4例，卵巢8例，胎盘2例。（6）临床分期：9例原发性生殖系统NHL患者中，Ⅰ期4例，Ⅲb期1例，Ⅳ期4例。（7）病理类型：20例淋巴造血组织肿瘤患者中，髓细胞肉瘤9例，髓性白血病侵犯胎盘2例，DLBCL 6例，CLL/SLL及黏膜相关淋巴组织淋巴瘤（MALToma）各1例，间变大细胞淋巴瘤（ALCL）1例。（8）免疫组化法检测：髓细胞肉瘤和髓性白血病为CD(43)、髓性过氧化物酶（MPO）呈阳性表达，NHL为CD(20)、配对盒基因5抗体（PAX5）呈阳性表达。（9）治疗及预后：淋巴造血组织肿瘤多采用手术切除辅以化疗。随访期内，20例淋巴造血组织肿瘤患者中，髓细胞肉瘤或髓性白血病5例死亡，NHL 2例死亡；13例子宫颈淋巴瘤样病变患者均健在。 结论： 临床病史、病理形态和免疫组化法检测对于诊断生殖系统的淋巴造血组织肿瘤具有重要作用，治疗采用手术治疗辅以化疗。子宫颈淋巴瘤样病变应与淋巴瘤鉴别，前者的预后良好。.
Keywords: Genital neoplasms, female; Leukemia, myeloid; Lymphoma, non-Hodgkin; Lymphoproliferative disorders.