Clinical diagnosis
Case 329
【Progress】
She was introduced to Universal Hospital for further management.
【Discussion】
Macrophages, dendric cells and Langerhans cells resident skin, digestive tract, bronchial tract, and vaginal tract directly facing toxic or non-toxic foreign body. They contribute to work as first-line defense for enemy. They engulf almost everything not self. Dendric cells and Langerhans cells not just engulf foreign body but also carry to present antigen, taken out foreign body to helper T cells and regulatory T cells which are directors of immune system. Namely, when foreign bodies are pathogenic, helper T cells stimulate B cells to create antibodies, and when they are not pathogenic, regulatory T cells work to suppress antibody creation, inducing to prevent allergy. Then, dendric cells and Langerhans cells are not mere phagocytes and carriers, but also own abilities to distinct enemies from not enemies. It is thought that when foreign bodies are pathogen, they carry the antigens to helper T cells and when they are not pathogen, they carry them to regulatory T cells (1-3). Both of dendrites and Langerhans cells own common dendric process at cellular surface. The difference between dendrites and Langerhans cells is whether it contains Birbeck granules whose functions are not clarified (1). Langerhans cell histiocytosis emerge from mutation of Langerhans cell based on different gene arrangement.
Langerhans cell histiocytosis is currently categorized into three types: unifocal (one system), multifocal (one system), multisystem (multi-organ): This disease was previously classified into eosinophilic granuloma (uniform), Hand-Schuller-Christian disease (diabetes insipidus, exophthalmos, bone lesion, multisystem) and Letterer-Siwe syndrome (dissemination with risk organ, multisystem).
Langerhans cell histiocytosis mainly appears in bone, skin, lung, spleen and bone marrow (1-3). The symptoms are rash, swelling and pain caused by prostaglandin secretion from histiocytes (3).
Langerhans cell histiocytosis appears in skull, pelvic bone, vertebra and long bone. Skull is the most susceptible followed by pelvic bone, vertebra and spine. Radiographically, it appears radiolucent area called punched-out or geographical. Differential diagnosis is listed as osteomyelitis, Ewing tumor and metastasis such as from neuroblastoma in children and lung cancer in adults (4, 5).
Langerhans cell histiocytosis is managed depending upon category, patient age and extent, with surgical curettage, resection, chemotherapy and radiation therapy. Five-year survivals are 100% and 100% in focal and multifocal types, 80% in multisystem type (6).
In our case, painful swollen mass at parietal bone whose radiography and CT depict osteolytic lesion, compatible with Langerhans cell histiocytosis. She is scheduled to receive treatment in university hospital.
【Summary】
We presented a twelve-year-old female for painful skull mass. Brain CT depicted osteolytic lesion at left parietal bone. She was introduced to university hospital based on diagnosis of Langerhans cell histiocytosis for further examination and treatment. It is borne in mind that Langerhans cell histiocytosis is categorized three types: unifocal (eosinophilic granuloma), multifocal and multisystem (Hand Schuller Christian disease, Lettere Siwe syndrome). The symptoms appear via prostaglandin secreted by histiocytes including Langerhans cell which works engulf foreign bodies and carry pathogen to helper T cells and carry non-pathogen to repressive T cell. They are managed by surgical curettage, radiotherapy and chemotherapy dependent upon patient age, category, and extent, inducing good prognosis especially on unifocal type.
【References】
1.Pathology and Genetics: Tumours of Haematopoietic and Lymphoid Tissues (World Health Organization Classification of Tumours). (2003)
2.Emile J-F, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–81.
3.Reisi N, et al. Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature. Orphanet Journal of Rare Diseases volume 16, Article number: 1 (2021)
4.Khung S, et al. Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 2013;4(5):569–79.
5.Schmidt S, Eich G, Geoffray A et al. Extraosseous Langerhans Cell Histiocytosis in Children. Radiographics. 2008;28(3):707-26
6.Kálmán J, et al. A unique manifestation of Langerhans cell histiocytosis: diagnostic and therapeutic considerations of atypical cases. ENT Ear Nose Throat J. 2018;97(4–5):E13–E17. PMID: 29940687.
2024.3.29