成人肺郎格罕细胞组织细胞增多症伴骨质增生:了解这种罕见的恶性肿瘤模拟

S.A. Smith, A. Franks, William D. Rollyson, D. Griswold, Tammy Bannister
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摘要

郎格罕细胞是形成人体抗原屏障的树突细胞。它们几乎出现在任何组织中,但最常见于皮肤、支气管树粘膜下层和其他粘膜。Langerhans细胞组织细胞增多症(LCH)是指这些细胞通过炎症和单克隆刺激的组合破坏其所在的组织。LCH的肺部变体涉及肺实质,并造成许多障碍,包括肺动脉高压、阻塞性和限制性肺病。除了肺部变异外,骨质受累还表现为肺部肿块和溶解性骨病变,这引发了恶性肿瘤的怀疑。早期认识这种罕见的病理是重要的,因为早期治疗在临床上是有益的。以下探讨一例成人肺郎格罕细胞组织细胞增多症伴骨受累的病例。
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Adult Pulmonary Langerhans Cell Histiocytosis with Osseous Involvement: understanding this rare mimic of malignancy
Langerhans cells are dendritic cells that form the antigenic barrier of the human body. They occur in nearly any tissue but are most prevalent in the skin, submucosa of the bronchial tree, and other mucosae. Langerhans Cell Histiocytosis (LCH) develops when these cells damage the tissues in which they reside through a combination of inflammatory and monoclonal stimulation. The pulmonary variant of LCH involves the lung parenchyma and creates many disturbances, including pulmonary hypertension and obstructive and restrictive lung disease. Osseous involvement, in addition to the pulmonary variant, presents with pulmonary masses and lytic bone lesions, which sparks suspicion for malignancy. Early recognition of this rare pathology is important as early treatment is clinically beneficial. The following explores a case of adult Pulmonary Langerhans Cell Histiocytosis with osseous involvement.
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