摘要目的 探讨毛细胞白血病(HCL)脾脏的临床病理学特征、诊断与鉴别诊断及脾切除预后.方法 回顾性分析15例HCL脾切除患者的临床资料,脾脏常规石蜡切片,HE及免疫组织化学染色(EliVision二步法)后光镜观察,并进行随访.结果 (1)15例患者,男11例,女4例,男:女为2.75:1,中位年龄47(36～68)岁.均以脾大为主要临床表现,B症状(发热、盗汗、体重减轻)不明显.多伴血红蛋白降低(80.0%)、血小板降低(60.0%)和白细胞升高(53.3%),少数全血细胞减少(20.0%).(2)特殊检查:外周血和骨髓毛细胞比例为(14.6±7.2)%和(47.3±23.8)%;骨髓细胞耐酒石酸酸性磷酸酶(TRAP)阳性率62.5%,咐醇酯(TPA)诱导附壁试验阳性率85.7%,毛细胞透射电镜核糖体板层复合体(RLC)检出率25%;骨髓活检HCL累及率100%.(3)15例患者脾脏重量(3012±1974)g,镜下白血病细胞呈弥漫性增生,淋巴滤泡完全消失或严重萎缩(14/15),红髓窦-索结构不清.3例可见"血湖"形成,白血病细胞胞质丰富淡染,免疫组织化学示CD45RA、CD20和PAX-5、CD25、CD11c、Annexin A1、cyclinD1阳性,CD3、CD43阴性.(4)随访13例目前均存活.随访时间5年以上的9例,10年以上的7例.结论 该病以中老年缓慢起病,脾大为首发症状,结合脾脏组织形态、免疫组织化学及相关检杳确诊.脾切除后可长期缓解.

abstractsObjective To investigate the clinicopathologic features, diagnosis,differential diagnosis and the prognosis of hairy cell leukemia (HCL). Methods Fifteen splenectomy specimens of HCL patients were investigated retrospectively using HE and immunohistochemistry in correlation with the follow-up information. Results (1) The male to female ratio was 2.75:1, age ranged from 36 to 68 years with a median of 47 years. The most consistent clinical feature at presentation was marked splenomegaly(100%). Other symptoms included anemia (80.0%), thrombocytopenia (60.0%), leucocytosis (53.3%), pancytopenia(20.0%) and the absence of B-symptom. (2)The proportion of hairy cells was (14.6± 7.2) % in periphery blood and (47.3±23.8) % in bone marrow. The positive rate of TRAP assay was 62.5% in bone marrow;85.7% for TPA test and the detection rate for RLC was 25% by transmission electric microscopy. The frequency of bone marrow involvement was 100%. (3)The average weight of 15 spleens was (3012±1974) g. The size of 6 spleens ranged from 16 cm×10 cm×5 cm to 32 cm×20 cm× 14 cm. The white pulp of spleen showed a characteristic atrophy feature or even absent due to leukemic infiltration, predominantly involving the red pulp with some sinusoidal pattern. "Blood pool" change was an infrequent feature (3/15 cases). The nuclei of leukemic cells were round (13 cases) or bean-shaped (2 cases), nucleoli inconspicuous or disappeared. The abundant cytoplasm and prominent cell border resulted in a "fried egg" appearance. By immunohistochemistry, leukemic cells were positive for CD45RA, CD20, PAX-5, CD25, CD11c, Annexin AI and cyclinD1, but negative for CD3 and CD43. (4)13 cases (86.7%) have been followed-up and all are alive. Among them, 9 cases are living well more than 5 years and 7 more than 10 years. Conclusions Splenomegaly is frequently the first manifestation of patients with HCL and occurred predominantly in the middle to elderly adults. Definite diagnosis of HCL requires a combined histological and immunohistochemical assessment of the splenectomy specimen, bone marrow biopsy and aspirate.