Karki Prasanna, Hirano Hirofumi, Yamahata Hitoshi, Fujio Shingo, Yonezawa Hajime, Iida Koji, Bohara Manoj, Oyoship Tatsuki, Hanaya Ryosuke, Arita Kazunori
Hiroshima J Med Sci. 2015 Dec;64(4):59-63.
Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. LCH has an excellent prognosis when treated with surgical resection, steroids and radiotherapy or chemotherapy. One of our patients is disease free at 7 year follow-up and one patient had regression of lesion on follow-up.
朗格汉斯细胞组织细胞增多症（LCH）是朗格汉斯细胞与炎症细胞（特别是嗜酸性粒细胞）混合增生，可表现为单系统（单灶性或多灶性）或多系统疾病。我们描述了我们两例眼眶和颅骨LCH的临床和组织学特征。两例均有单灶性颅骨侵蚀性病变且有外伤史。典型的组织学特征包括大量具有不同程度巨细胞形成的组织细胞和散在的嗜酸性粒细胞。通过CD1a和S100免疫组化证实了朗格汉斯细胞的存在。LCH经手术切除、类固醇及放疗或化疗治疗后预后良好。我们的一名患者在7年随访时无疾病，另一名患者随访时病变消退。
