Langerhans Cell Histiocytosis without Eosinophilia and Lytic Lesions of Bone: A Rare Disease with Unusual Presentation

S. Chauhan, Sunita Sharma, Smita Singh, S. Mukherjee
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Abstract

LCH is a disease of abnormal clonal proliferation of langerhans cell of the bone marrow derived monocyte-macrophage lineage. Cells with characteristic coffee bean / grooved / indented nuclei with a background of histiocytes, lymphocytes, eosinophils and other inflammatory cells are seen. Clinical presentation varies from unifocal unisystem to multifocal unisystem to multifocal multisystem. We present a rare case of multisystem LCH involving high risk organs in a two and a half year old child. presented with the chief complaints of fever and red raised maculopapular lesions on trunk, palm, forehead and scalp for one and a half yrs with hepatosplenomegaly. There were no lytic lesions of the bones. Hematological parameters showed anaemia with thrombocytopenia while Bone Marrow Aspirate showed increase in histiocytic cell with some cells showing characteristic coffee bean, cleaved irregularly contoured nuclei. However, eosinophilia was not seen. Sections from the skin lesions showed cells with similar morphology without usually accompanied eosinophilia. The cells were CD1a and S100 positive. So a FINAL DIAGNOSIS of langerhans cell histiocytosis was made. The emphasis here lies on thorough analysis of the clinical presentations, imaging studies and scrupulous histomorphological and immunohistochemical examination, so that early diagnosis and timely intervention is ensured in such cases where the characteristic features like lytic lesions of bone and eosinophilia is not found.
朗格汉斯细胞组织细胞增多症无嗜酸性粒细胞增多和骨溶解性病变:一种罕见的疾病与不寻常的表现
LCH是一种来源于骨髓的单核-巨噬细胞谱系的朗格汉斯细胞异常克隆增生的疾病。细胞具有特征性的咖啡豆/沟槽/凹痕核,背景有组织细胞、淋巴细胞、嗜酸性粒细胞和其他炎症细胞。临床表现从单焦点单系统到多焦点单系统到多焦点多系统不等。我们报告一例罕见的涉及高危器官的多系统LCH病例,患者为两岁半的儿童。主诉为发热,躯干、手掌、前额和头皮上红色凸起的黄斑丘疹病变一年半,伴肝脾肿大。骨头没有溶解性损伤。血液学指标显示贫血伴血小板减少,骨髓抽吸显示组织细胞增多,部分细胞呈特有的咖啡豆状,细胞核呈不规则形状。但未见嗜酸性粒细胞增多。皮肤病变切片显示细胞形态相似,通常不伴有嗜酸性粒细胞增多。细胞CD1a和S100阳性。最终诊断为朗格汉斯细胞组织细胞增多症。重点在于充分分析临床表现、影像学检查,仔细进行组织形态学和免疫组化检查,在未发现骨溶解性病变、嗜酸性粒细胞增多等特征性表现的情况下,确保早期诊断和及时干预。
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