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Case Report
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| Langerhans cell histiocytosis in the right scapula in a young man |
| Georgios Tsirakis1, Maria Kaparou2, Peggy Kanellou2, Georgios Kontakis3, Michael Alexandrakis4 |
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1Consultant Haematologist, Department of Haematology, University Hospital of Heraklion, Crete, Greece.
2Resident in Haematology, Department of Haematology, University Hospital of Heraklion, Crete, Greece. 3Associate Professor of Orthopedics, Department of Orthopedics and Traumatology, University Hospital of Heraklion, Crete, Greece. 4Associate Professor of Haematology, Department of Haematology, University Hospital of Heraklion, Crete, Greece. |
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doi:10.5348/ijcri-2012-01-83-CR-5
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Address correspondence to: Michael Alexandrakis University Hospital of Heraklion Department of Haematology P.O. box 1352, Heraklion, Crete Greece, P.C. 71110 Phone: 00302810392425 Fax: 00302810392426 Email: alexandm@med.uoc.gr |
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| How to cite this article: |
| Tsirakis G, Kaparou M, Kanellou P, Kontakis G, Alexandrakis M. Langerhans cell histiocytosis in the right scapula in a young man. International Journal of Case Reports and Images 2012;3(1):16-19. |
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Abstract
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Introduction:
Langerhans cell histiocytosis (LCH) is a proliferative histiocytic disorder with a variable clinical manifestations. It may be localized or disseminated. The aetiology and pathogenesis of the disease are unknown. Various mechanisms regarding the aberrant expression of chemokines' receptors or dysregulation of chemokine production in the lesions, probably take part in the pathophysiology of the disease.
Case Report: We report a rare case of Langerhans cell histiocytosis in the right scapula. A 28-year-old man presented with pain in right shoulder. A localized osteolysis of glenoid with disruption of the cortex, accompanied by extensive bone edema was detected with CT and MRI, whereas Tc-99m bone scan revealed increased concentration of the radionuclide in the affected area. The bone lesion biopsy revealed Langerhans cell histiocytosis (CD1a+, S-100+, CD68+), and a palpable spleen was also revealed. After the removal of the lesion, he was treated with systematic chemotherapy and the patient remains with no active disease for four years. Conclusion: Langerhans cell histiocytosis is a disease of histiocytes with multiple pathophysiologic aetiologies. The diagnosis is based on morphological and immunohistochemical characteristics. The clinical manifestations vary depending on the organs or systems affected. It most frequently affects bone, skin and pituitary and less commonly haematopoietic system, spleen, lungs and central nervous system. In the bones, the lesions are usually localized to the skull, particularly in the jaw, long tubular bones, vertebrae, pelvis and ribs. Scapula is a very rare site for LCH. This is one of very few case reports of LCH in the scapula. | |
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Key Words:
Langerhans cell histiocytosis, Scapula, Osteolysis
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Author Contributions:
Georgios Tsirakis - Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be publishe. Maria Kaparou - Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published Peggy Kanellou - Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published Georgios Kontakis - Acquisition of data, Drafting the article, Critical revision of the article, Final approval of the version to be published Michael G Alexandrakis - Conception and design, Drafting the article, Critical revision of the article, Final approval of the version to be published |
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Guarantor of submission:
The corresponding author is the guarantor of submission. |
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Source of support:
None |
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Conflict of interest:
Authors declare no conflict of interest. |
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Copyright:
© Georgios Tsirakis et al. 2012; This article is distributed the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any means provided the original authors and original publisher are properly credited. (Please see Copyright Policy for more information.) |
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