| Autor: |
Young Kwon Koh, Su Hyun Yoon, Sung Han Kang, Hyery Kim, Ho Joon Im, Pyeong Hwa Kim, Ah Young Jung, Kyung-Nam Koh |
| Jazyk: |
English<br />Korean |
| Rok vydání: |
2022 |
| Předmět: |
|
| Zdroj: |
Clinical Pediatric Hematology-Oncology, Vol 29, Iss 2, Pp 97-101 (2022) |
| Druh dokumentu: |
article |
| ISSN: |
2233-5250 |
| DOI: |
10.15264/cpho.2022.29.2.97 |
| Popis: |
Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by heterogenous lesions infiltrated with CD1a+/CD207+ cells. Although LCH has a relatively good prognosis, the prognosis for patients with LCH refractory to standard chemotherapy is poor. Neurodegenerative LCH (ND-LCH) is a central nervous system complication of LCH that is characterized by progressive radiological and clinical abnormalities. Symptomatic ND-LCH is difficult to treat and therefore has a poor prognosis. A two-year-old boy presented with a scalp mass. Biopsy confirmed LCH. Whole-body imaging revealed LCH involvement in multiple bones of the skull, facial bones, and lungs. Prednisolone and vinblastine chemotherapy was initiated. One-year post-treatment, most of the lesions in the bones and lung nodules disappeared, and chemotherapy was discontinued. New neurodegenerative lesions appeared 4 months after chemotherapy was discontinued. Second-line chemotherapy using cytarabine, vincristine, and prednisolone was initiated. However, neurological manifestations of ND-LCH worsened post second-line treatment, and the treatment was switched to cytarabine and cladribine. Despite third-line chemotherapy, the lesions progressed, and neurological deficits worsened. After identifying BRAF V600E mutation in the tumor tissue using next-generation sequencing, cytotoxic chemotherapy was discontinued and vemurafenib treatment was initiated. One-year post-vemurafenib therapy, ND-LCH manifestations regressed, and the patient experienced neurological improvement. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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