Freitas José Guilherme, Jorge Ana, Rei Daniel, Graça Joana
Department of Internal Medicine, Centro Hospitalar de Lisboa Ocidental EPE, Lisboa, Portugal.
Department of Hematology, Centro Hospitalar de Lisboa Ocidental EPE, Lisboa, Portugal.
BMJ Case Rep. 2018 Jan 10;2018:bcr-2017-222306. doi: 10.1136/bcr-2017-222306.
We report a case of a 90-year-old man with hypereosinophilia, lymphadenopathies and skin lesions, namely lichenification and pruritus. An aetiological investigation was performed, and a bone marrow (BM) biopsy and aspirate showed a hypercellular marrow with hypereosinophilia without dysmorphia or abnormal elements, and the BM and inguinal node's immunophenotyping denied any presence of abnormal lymphoid cell population. The inguinal node biopsy revealed a multinodular proliferation of large cells S100 and CD1a, and a diagnosis of Langerhans cell histiocytosis was made. The hypereosinophilia and skin lesions were managed with corticotherapy with substantial improvement of cutaneous lesions and lymphadenopathies and normalisation of eosinophil count. Finally, to define if it is a single or multisystem disease, a skin biopsy will be necessary.
我们报告一例90岁男性患者,患有嗜酸性粒细胞增多症、淋巴结病和皮肤病变,即苔藓样变和瘙痒。进行了病因学调查,骨髓活检和穿刺显示骨髓细胞增多伴嗜酸性粒细胞增多,无畸形或异常成分,骨髓和腹股沟淋巴结的免疫表型分析排除了任何异常淋巴细胞群的存在。腹股沟淋巴结活检显示S100和CD1a大细胞呈多结节性增生,诊断为朗格汉斯细胞组织细胞增多症。嗜酸性粒细胞增多症和皮肤病变采用皮质激素治疗,皮肤病变和淋巴结病明显改善,嗜酸性粒细胞计数恢复正常。最后,为了确定这是单系统还是多系统疾病,有必要进行皮肤活检。