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Acquired Disorders of the Neck
Published in Raymond W Clarke, Diseases of the Ear, Nose & Throat in Children, 2023
Langerhans cell histiocytosis (LCH) occurs mainly in young children (under the age of 5 years). It is a multisystem disease, again of unknown aetiology, associated with unexplained fever, malaise and weight loss. LCH, known in the past as Letterer–Siwe disease or eosinphilic granulomatosis, is characterised by the presence of lesions – including in the cervical nodes – which have a distinctive histological appearance and may initially be mistaken for lymphoma. It can run a very aggressive course with a significant mortality (10%). Children need careful work-up and should be referred to a paediatric oncologist as some will be considered for chemotherapy.
Granulomatous Diseases
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Albert Alhatem, Robert A. Schwartz, Muriel W. Lambert, W. Clark Lambert
Overview: Xanthogranuloma (XG) is a moderately uncommon, benign, proliferative histiocytic disorder, which originates from dermal factor XIIIa positive dendrocytes. It is the most common of the non-Langerhans cell histiocytosis, occurring predominantly in young children.
The locomotor system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Langerhans’ cell histiocytosis may occur at any age or site, but most commonly arises in children and young adults with one or more lesions in the skull, long bones, vertebrae, and pelvis. Well-defined lytic lesions are seen, although in long bones cortical erosion and pathological fracture may occur, suggesting malignancy. Vertebral involvement may lead to bony collapse and a flat dense vertebra (vertebra plana). Histologically, groups of pale-staining Langerhans’ cells are mixed with numerous eosinophils. Patients with solitary or few lesions may be cured by surgery or low doses of radiotherapy, or the lesions may heal spontaneously. It is important to ascertain whether there is systemic involvement because this worsens the prognosis. Many cases are associated with mutation of the BRAF gene which can be targeted by BRAF inhibitors.
Iris Juvenile Xanthogranuloma Presenting with Hypopyon
Published in Ocular Immunology and Inflammation, 2022
Mine Esen Baris, Mukaddes Damla Ciftci, Melis Palamar, Suzan Guven Yilmaz
A 45-day-old infant was referred to our clinic for unilateral hypopyon. The mother indicated noticing a whitish color change in the left eye multiple times that resolved rapidly. However, at the time of consultation, the whiteness was more intense and was retained for almost 2 weeks. The baby was examined under general anesthesia and found to have hypopyon that gave a level of about 2 mm in the left eye. No pathology was observed in the visible areas of the iris. Intraocular pressure (IOP) was 13 mmHg (Tono-Pen®, TP; Reichert, New York, USA) and the fundus examination was completely normal. Anterior and posterior segment examinations of the right eye were normal and IOP was 12 mmHg. B-scan ultrasound examinations of both eyes were within normal limits. Cranial and orbital MRI scans were reported to be normal as well. Complete blood count, along with the erythrocyte sedimentation rate and C-reactive protein levels, was within normal limits. Langerhans cell histiocytosis and granulomatous infections (tuberculosis and syphilis) were investigated. Syphilis was excluded by serological tests and interferon gamma release assay (IGRA) was applied for exclusion of tuberculosis. Examinations of the infant by the pediatrician and the blood tests along with cranial and orbital MRI revealed no pathology; therefore, Langerhans cell histiocytosis was also excluded.
Chronic Recurrent Multifocal Osteomyelitis (CRMO): A Study of 12 Cases from One Institution and Literature Review
Published in Fetal and Pediatric Pathology, 2022
Eric Chang, Jasmine Vickery, Nadeen Zaiat, Eman Sallam, Abdul Hanan, Scott Baker, Mohamed Alhamar, Janet Poulik, Ereny Demian, Bahig M Shehata
Our biopsies revealed no associated soft tissue response. No microorganisms were identified, confirmed by negative bacterial, fungal, and mycobacterial cultures. All lesions were negative for CD1a, precluding Langerhans cell histiocytosis. The final diagnosis was determined collectively by the radiologist, pathologist, pediatrician, and infectious disease physician after correlating the clinical presentation, radiological, and bone biopsy findings. Once the correct diagnosis was rendered patients were treated with anti-inflammatory medication and steroids. Treatment plan and duration varied, but had an average duration of 6 months. Our patient cohort was followed over a span of 7 months to 6.5 years. 9 of these patients responded to therapeutic interventions with complete healing. The other 3 had recurrent lesions, including at the same sites, after a variable length of remission.
Clinical and prognostic characteristics of 95 cases of Langerhans cell histiocytosis in children: a single-institute experience from 2013 to 2020
Published in Annals of Medicine, 2021
Xue Tang, Ju Gao, Zhi-gui Ma, Xia Guo, Qiang Li, Zhi Wan, Jing-jing Sun
Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder in children, with a prevalence of approximately 5–9 cases per 1 million children under the age of 15 years [1,2]. Recently, LCH has been defined as a disorder driven by misguided myeloid differentiation [3] and identified with mutually exclusive somatic mutations in mitogen-activated protein kinase (MAPK) pathway genes in approximately 75% of LCH cases [4]. The extent of the disease or organs and systems involved form the basis of the LCH clinical classification system: single-system LCH (SS-LCH) with one organ/system involved (uni- or multifocal SS-LCH) and multisystem LCH (MS-LCH) with two or more organs/systems involved, stratified by risk organ (RO) involvement (high (RO+) or low (RO–) risk groups) [5]. The single site SS-LCH usually requires local therapy or observation, while the current standard of frontline treatment in multifocal LCH is empirically derived chemotherapy [5–7].