Malignant histiocytosis X. A distinct clinicopathologic entity
Malignant histiocytosis X. A distinct clinicopathologic entity
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恶性组织细胞增多症 X. 一个独特的临床病理实体
作者：
J. Ben;A. Bailey;N. Azumi;G. Delsol;R. Stroup;K. Sheibani;H. Rappaport
X 组织细胞增多症 (HX) 的形态学特征是朗格汉斯细胞 (LC) 增殖，并且通常具有惰性、慢性病程。为了确定是否存在恶性组织细胞增生症 X 的独特临床病理实体，作者检查了 31 名 HX 患者的组织，并将其分为四类。 A 组（19 名患者）的形态学特征为良性 LC，病程惰性。男女（M：F）比例为10：9，平均年龄为21岁（范围2个月至60岁）。该组的免疫表型为S-100+、波形蛋白+、LN-2+、LN-3+、溶菌酶-、LCA-、Leu-M1-。 B 组（三名患者）的 LC 看似良性，但临床病程却呈侵袭性。所有患者均为男性，平均年龄 47 岁（范围 3 岁至 72 岁）。涉及的器官包括肝脏、脾脏、心脏、胸腺、肺、肾和胰腺。免疫表型与 A 组相同。C 组（两名患者）具有非典型且恶性的 LC，但临床病程相对良性。患者年龄分别为 4 岁和 65 岁，其中 1 名女性和 1 名男性患者。两名患者的细胞均为 S-100+、波形蛋白+、LN-2+、LN-3+ 和 LCA-。 D 组（七名患者）的特点是非典型且恶性的 LC 以及侵袭性的临床病程。平均年龄为 25 岁（范围，先天性至 54 岁），其中 1 名女性和 6 名男性患者。涉及的器官有胸腺、肺、脾、肝、肾、脑、心脏、胰腺、胃和肌肉。在两名患者体内发现了伯贝克颗粒，一名有新鲜组织的患者为 CD1+。典型的免疫表型是S-100+、波形蛋白+、LN-2+、LN-3+、Leu-M1-、溶菌酶-。我们的研究结果表明，（1）确实存在一种恶性 HX 的独特临床实体，其形态学特征为恶性 LC，临床特征为男性占主导地位、非典型器官受累和侵袭性临床病程； (2) LC 的形态外观不能完美预测 HX 的临床严重程度。
Histiocytosis X (HX) is characterized morphologically by a proliferation of Langerhans' cells (LC), and most often has an indolent, chronic course. To determine whether a distinct clinicopathologic entity of malignant histiocytosis X exists, the authors examined tissues from 31 patients with HX and divided them into four categories. Group A (19 patients) was characterized morphologically by benign‐appearing LC and had an indolent course. The male:female (M:F) ratio was 10:9, and the mean age was 21 years (range, 2 months to 60 years). The immunophenotype of this group was S‐100+, vimentin+, LN‐2+, LN‐3+, lysozyme−, LCA−, Leu‐M1−. Group B (three patients) had benign‐appearing LC, yet had an aggressive clinical course. All patients were male, with a mean age of 47 years (range, 3 years to 72 years). Organs involved included the liver, spleen, heart, thymus, lung, kidney, and pancreas. The immunophenotype was the same as for Group A. Group C (two patients) had atypical and malignant appearing LC, yet a relatively benign clinical course. The ages were four and 65 years, with one female and one male patient. In both patients, the cells were S‐100+, vimentin+, LN‐2+, LN‐3+, and LCA−. Group D (seven patients) was characterized by atypical and malignant‐appearing LC and an aggressive clinical course. The mean age was 25 years (range, congenital to 54 years) with one female and six male patients. Organs involved were the thymus, lungs, spleen, liver, kidney, brain, heart, pancreas, stomach, and muscle. Birbeck granules were found in two patients, and the one patient on which fresh tissue was available was CD1+. The typical immunophenotype was S‐100+, vimentin+, LN‐2+, LN‐3+, Leu‐M1−, lysozyme—. The results of our study indicate that (1) a distinct clinical entity of malignant HX, characterized morphologically by malignant‐appearing LC and clinically by male predominance, atypical organ involvement, and an aggressive clinical course, does exist; and (2) the morphologic appearance of the LC is an imperfect predictor of the clinical severity of HX.
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酶免疫组织化学：技术方面。
影响因子：
2.3
作者：
Sheibani,K;Tubbs,RR
通讯作者：
Tubbs,RR
朗格汉斯细胞组织细胞增多症的抗原表型：一项免疫组织化学研究证明了 LN-2、LN-3 和波形蛋白的价值。
影响因子：
3.3
作者：
Azumi,N;Sheibani,K;Swartz,WG;Stroup,RM;Rappaport,H
通讯作者：
Rappaport,H
DOI：
--
发表时间：
1985
期刊：
The American journal of pathology
影响因子：
--
作者：
Wood,GS;Turner,RR;Shiurba,RA;Eng,L;Warnke,RA
通讯作者：
Warnke,RA
组织细胞增多症-X。
DOI：
10.1056/nejm198101153040304
发表时间：
1981
期刊：
The New England journal of medicine
影响因子：
--
作者：
Osband,ME;Lipton,JM;Lavin,P;Levey,R;Vawter,G;Greenberger,JS;McCaffrey,RP;Parkman,R
通讯作者：
Parkman,R
外周 T 细胞淋巴瘤：与形态进展相关的免疫学和细胞动力学观察。
影响因子：
20.3
作者：
Winberg,CD;Sheibani,K;Krance,R;Rappaport,H
通讯作者：
Rappaport,H