Acta Medica Okayama 73巻 1号
2019-02 発行

Remission of Congenital Multi-system Type Langerhans Cell Histiocytosis with Chemotherapy

Tamefusa, Kosuke Department of Pediatrics, Okayama University Hospital
Ishida, Hisashi Department of Pediatric Hematology and Oncology, Okayama University Hospital
Washio, Kana Department of Pediatrics, Okayama University Hospital
Ishida, Toshiaki Department of Hematology/Oncology, Hyogo Prefectural Children’s Hospital
Morita, Hirosuke Department of Pediatrics, Kagawa Prefectural Central Hospital
Shimada, Akira Department of Pediatric Hematology and Oncology, Okayama University Hospital
Publication Date
2019-02
Abstract
Patients with multi-system (MS)-type langerhans cell histiocytosis (LCH) show poor outcomes, especially congenital MS LCH cases were shown in high mortality rate. We experienced a congenital case of MS LCH with high risk organs, who needed intensive respiratory support after birth. Even though intensive chemotherapy was discontinued, this patient’s lung LCH lesions gradually became reduced and his respiratory condition recovered; therefore, we restarted and completed maintenance chemotherapy. The patient maintained complete remission for more than 4 years after the end of chemotherapy. Our case suggests that congenital MS LCH even with severe organ involvement can be treated successfully with chemotherapy.
Document Type
Case Report
Keywords
Langerhans-cell histiocytosis
congenital
multisystem type
Link to PubMed
Thumnail 73_1_61.pdf 2.55 MB