Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report
Langerhans cell histiocytosis (LCH) is a rare disorder, especially among adults, characterized by abnormal accumulation of dendritic histiocytes in various tissues, presenting as either single- or multi-system disease. In adults, spinal involvement is less common than long bone, while central nervou...
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| Language: | English |
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Elsevier
2025-03-01
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| Series: | Radiology Case Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1930043324013360 |
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| author | Yaman M․ Alahmad Omar Al Mukdad Ahmad Huneity Sarah Sayed Renan Adam Alaa Al-Taie |
| author_facet | Yaman M․ Alahmad Omar Al Mukdad Ahmad Huneity Sarah Sayed Renan Adam Alaa Al-Taie |
| author_sort | Yaman M․ Alahmad |
| collection | DOAJ |
| description | Langerhans cell histiocytosis (LCH) is a rare disorder, especially among adults, characterized by abnormal accumulation of dendritic histiocytes in various tissues, presenting as either single- or multi-system disease. In adults, spinal involvement is less common than long bone, while central nervous system manifestations, such as pituitary gland enlargement and stalk thickening, affect about a quarter of adult patients and may lead to significant endocrine disorders. Salivary gland involvement is another extremely rare manifestation of LCH. We report a 46-year-old lady who presented with back pain along with sensory and motor deficits in the lower limbs. Her blood pressure was elevated. Computed tomography angiography spotted a T6 vertebral body compression fracture. Magnetic resonance of the spine showed T6 vertebral body pathological collapse with epidural soft tissue component compressing the spinal cord. Other imaging modalities showed features suggestive of central nervous system and salivary gland involvement. She underwent T4-T8 pedicle screws fixation with T6 decompressive laminectomy and biopsy of the epidural lesion, which revealed histopathological features of LCH. This is an extremely rare case of adult-onset LCH with multi-system involvement: musculoskeletal, salivary and central nervous systems. This case could serve as a crucial reference for both clinicians, radiologists and researchers, enriching the existing knowledge base on adult-onset LCH. |
| format | Article |
| id | doaj-art-1cd2be247cd242bcb8f244f6f46aa0db |
| institution | Kabale University |
| issn | 1930-0433 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj-art-1cd2be247cd242bcb8f244f6f46aa0db2025-01-18T05:03:58ZengElsevierRadiology Case Reports1930-04332025-03-0120312891293Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case reportYaman M․ Alahmad0Omar Al Mukdad1Ahmad Huneity2Sarah Sayed3Renan Adam4Alaa Al-Taie5Radiology Department, Hamad Medical Corporation, Doha, Qatar; Corresponding author.Radiology Department, Hamad Medical Corporation, Doha, QatarRadiology Department, Hamad Medical Corporation, Doha, QatarPathology Department, Hamad Medical Corporation, Doha, QatarRadiology Department, Hamad Medical Corporation, Doha, QatarRadiology Department, Hamad Medical Corporation, Doha, QatarLangerhans cell histiocytosis (LCH) is a rare disorder, especially among adults, characterized by abnormal accumulation of dendritic histiocytes in various tissues, presenting as either single- or multi-system disease. In adults, spinal involvement is less common than long bone, while central nervous system manifestations, such as pituitary gland enlargement and stalk thickening, affect about a quarter of adult patients and may lead to significant endocrine disorders. Salivary gland involvement is another extremely rare manifestation of LCH. We report a 46-year-old lady who presented with back pain along with sensory and motor deficits in the lower limbs. Her blood pressure was elevated. Computed tomography angiography spotted a T6 vertebral body compression fracture. Magnetic resonance of the spine showed T6 vertebral body pathological collapse with epidural soft tissue component compressing the spinal cord. Other imaging modalities showed features suggestive of central nervous system and salivary gland involvement. She underwent T4-T8 pedicle screws fixation with T6 decompressive laminectomy and biopsy of the epidural lesion, which revealed histopathological features of LCH. This is an extremely rare case of adult-onset LCH with multi-system involvement: musculoskeletal, salivary and central nervous systems. This case could serve as a crucial reference for both clinicians, radiologists and researchers, enriching the existing knowledge base on adult-onset LCH.http://www.sciencedirect.com/science/article/pii/S1930043324013360Langerhans cell histiocytosisBack painNeurologic deficitsMultisystem involvementDiagnostic imagingHistopathology |
| spellingShingle | Yaman M․ Alahmad Omar Al Mukdad Ahmad Huneity Sarah Sayed Renan Adam Alaa Al-Taie Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report Radiology Case Reports Langerhans cell histiocytosis Back pain Neurologic deficits Multisystem involvement Diagnostic imaging Histopathology |
| title | Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report |
| title_full | Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report |
| title_fullStr | Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report |
| title_full_unstemmed | Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report |
| title_short | Adult-onset Langerhans cell histiocytosis with multisystem involvement: A rare case report |
| title_sort | adult onset langerhans cell histiocytosis with multisystem involvement a rare case report |
| topic | Langerhans cell histiocytosis Back pain Neurologic deficits Multisystem involvement Diagnostic imaging Histopathology |
| url | http://www.sciencedirect.com/science/article/pii/S1930043324013360 |
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