ALK阳性组织细胞增生症5例临床病理分析及文献复习
ALK positive histiocytosis:a clinicopathological analysis of five cases and review of literature
张燕林 1谢建兰 1周小鸽1
作者信息
- 1. 首都医科大学附属北京友谊医院病理科,北京 100050
- 折叠
摘要
目的 探讨ALK阳性组织细胞增生症临床病理特征.方法 总结5例ALK阳性组织细胞增生症的临床、形态和免疫组化结果,并随访.结果 男性2例,女性3例.年龄2个月到21岁.病程2周到5个月.症状主要①感觉和活动受限,皮肤结节,血小板和血红蛋白减少等.②低倍见小淋巴细胞及淡染组织细胞.高倍组织细胞呈卵圆形细胞核、嗜碱性核仁和嗜酸性胞质.可见侵入表皮现象.3例可见坏死.③免疫组化CD68、CD163和ALK阳性.④随访4～26个月,平均随访15.4个月.所有病例均显示ALK基因异常,包括4例KIF5B-ALK融合和1例CLTC-ALK融合.结论 ALK阳性组织细胞增生症具有独特的临床和组织形态学谱系,综合形态、免疫组化和分子检测有助于准确诊断和治疗后评估.
Abstract
Objective To analysis the clinical and pathological characteristics of ALK positive histiocytosis.Methods The clinical,morphological,and immunohistochemical results of 5 cases of ALK positive histiocytosis were summarized and followed up.Results There were 2 males and 3 females.Age ranged from 2 months to 21 years old.The course of the disease was 2 to 5 months.Symptoms mainly included sensory and activity disorders,skin nodules,decreased platelets and hemoglobin,etc.At low magnification,small lymphocytes and light stained histiocyte were seen.High magnification showed histiocyte had oval shaped nuclei,basophilic nucleoli and eosinophilic cytoplasm.Epidermotropic histocytes were noted.Three cases showed necrosis.Immunohistochemical staining was positive for CD68,CD163,and ALK.Follow-up for 4 to 26 months,with an average follow-up of 15.4 months.All cases showed ALK gene abnormalities,including four cases of KIF5B-ALK fusion and one of CLTC-ALK fusion.Conclusion ALK positive histiocytosis has a unique clinical and histological spectrum,and comprehensive morphology,immunohistochemistry and molecular detection are helpful for accurate diagnosis and post treatment evaluation.
关键词
组织细胞增生症/ALK/组织病理/中枢神经系统Key words
Hystiocytosis/Araplastic lymphoma kinase/Histopathology/Central nervous system引用本文复制引用
出版年
2024
NETL
NSTL
万方数据