Diagnosis and treatment of Langerhans Cell Histiocytosis with bone lesion in pediatric patient: A case report
| Autor: | Achmad Fauzi Kamal, Andi Praja Wira Yudha Luthfi |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
medicine.medical_specialty
Exophthalmos business.industry Osteomyelitis General Medicine medicine.disease Dermatology 03 medical and health sciences Langerhans cell hystiocytosis 0302 clinical medicine Langerhans cell histiocytosis Eosinophilic granuloma 030220 oncology & carcinogenesis Case report Diabetes insipidus Orthopedic surgery medicine Etiology 030211 gastroenterology & hepatology Surgery Bone lesion Medical diagnosis medicine.symptom business |
| Zdroj: | Annals of Medicine and Surgery |
| ISSN: | 2049-0801 |
| DOI: | 10.1016/j.amsu.2019.07.030 |
| Popis: | Background Langerhans cell histiocytosis (LCH) is a rare group of disorders without a well understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent. Methods We deliver a case of a 10-month-old boy who consulted to our department. Previously misdiagnosed as a Kawasaki syndrome, TORCH, and osteomyelitis. He had undergone several examinations and had been discussed in clinocipathological conference (CPC) to narrow down the diagnosis. Result After serial examinations, the diagnosis of Langerhans Cell Histiocytosis was confirmed and chemotherapy was initiated. And after 6 cycles of chemotherapy, with 1-week interval of each therapy, the clinical appearance of this patient significantly improved. Conclusion Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses for the orthopedic surgeon. By doing a comprehensive examination, it is possible to narrowing down the diagnosis and planning the accurate treatment. Highlights • Langerhans cell histiocytosis (LCH), also known as histiocytosis X, is a rare disorder resulting from an abnormal clonal proliferation of macrophages. • Despite major advances in our understanding and management of LCH, it remains one of the most challenging diagnoses. • In diagnosing LCH a thorough physical examination, Laboratory studies, and radiological studies are mandatory. • Many different treatments for multisystem LCH, from minimal therapy to intensive chemotherapy, have been tried. • Vinblastine and etoposide, have been identified as effective single agents. Both have activity against diverse disorders of the monocyte-macrophage system and combination chemotherapy including them had promising results in multisystem LCH. |
| Databáze: | OpenAIRE |
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