先天性白血病临床病理学分析(附6例尸检报告)

The Clinical and Pathologic Analysis of Congenital Leukemia(Attached Six Autopsy Reports)

目的 探讨先天性白血病的病理临床特点 .方法 收集 6例死亡病例 ,进行尸检及临床分析 .结果 6例检查中 ,肿瘤细胞广泛侵犯内脏器官 ,心、肝、脾、肺、肾和消化道的有 6例 ;骨髓、淋巴结、胸腺、胰、肾上腺有 5例 ;脑和甲状腺 4例 ;膀胱 3例 ;脑膜和皮肤 2例 ;舌咽、睾丸、子宫各 1例 .6例中急淋占 4例 ,急非淋 (急单 ) 2例 ;免疫分型 4例急淋全为T细胞性 ,表现为CD4 5RO(+)、CD3(+)表达 ;2例急非淋 ;1例CD6 8(+)、Lyso(+)表达 ,诊断为急单可能性大 (本例生后 1小时死亡 ,临床未及血液学检查、FAB分类 ) ;另 1例为早期病例 ,骨髓涂片FAB分型M5 ,未进行免疫检测分型 .结论 先天性白血病在尸检材料中见肿瘤细胞呈广泛性内脏器官浸润 ,并且血管腔内瘤栓形成 ,沿血道传播全身 .本病有发病早、病情急而凶险、治疗困难预后差、早期死亡的特点 .

Objective Congenital leukemia is very rare. We analyzed six death cases of the disease through autopsy. Results Tumor cells widely infiltrated splanchan organs. All cases can see neoplastic cells in heart,liver,spleen,lung,kidney and digestive tract.5 cases in Bone marrow,lymph nodes,thymus,pancreas and adrenal gland.4 cases in brain and thyroid. 3 in bladder.2 in meninges and skin. 1 in glosspharyngeum, testis and uterus respectively. 4 cases were acute lymphocytic leukemia(ALL), 2 were acute myeloid leukemia(acute monocytic leukemia). Immunohistochemically, 4 cases of ALL were positive for CD45RO and CD3, that was T lymphocytic cell immunophenotyping. 1 case of 2 AML was positive for CD68 and Lyso, so the diagnosis may be acute monocytic leukemia. The case had no time to do hematologic research and FAB study, because it survived only one hour after birth. The other was an old case, its bone marrow smear showed FAB-M5, and didn't do immunohistochemistry study.Conclusion Neoplastic cells widely infiltrated splanchan organs in autopsy cases of congenital leukemia, and even can see tumor thrombus in vessels, and metastase through blood. Congenital leukemia is a very early and dangerous disease, and it is difficult for treatment and has a bad prognosis.