成人朗格汉斯细胞组织细胞增生症3例并文献复习

目的 探讨成人朗格汉斯细胞组织细胞增生症(LCH)的临床表现、病理特点、治疗和预后.方法 回顾分析3例成人朗格汉斯细胞组织细胞增生症并复习相关文献.结果 3例LCH患者分别为39岁女性、36岁男性、56岁女性,临床受累脏器包括耻骨(1/3)、髂骨(1/3)、额骨(1/3)、股骨(1/3)、肋骨(1/3)、皮肤(1/3)、淋巴结(1/3),免疫组化为Cdla(+)、S-100(+),经联合化疗后,2例患者获得完全缓解,另1例多系统LCH患者反应较差,仍在进一步治疗观察中.结论 成人LCH少见,临床表现多样,易误诊、漏诊,治疗需个体化.

Adult Langerhans cell histiocytosis: A report of 3 cases and literature review

Objective To study the clinical manifestations,pathologic characteristics,treatment and prognosis of adult Langerhans cell histiocytosis(LCH).Methods Three adult LCH patients were retrospectively analyzed with related literature reviewed.Results The 3 LCH patients were a female at the age of 39 years,a male at the age of 36 years,and a female at the age of 56 years with their pubis(1/3),iliac(1/3),frontal bone(1/3),thigh bone(1/3),ribs(1/3),skin(1/3),lymph nodes(1/3) involved.Immunohistochemistry was positive for CD1a and S-100.After combined chemotherapy,2 patients achieved complete remission and the other patient with multiple systems involved had a poor response and needed further treatment and observation.Conclusion Adult LCH is a rare disease with various clinical manifestations.It is easy to be misdiagnosed and missed,and should be treated according to the condition of each patient.