International Journal of Hematology and Oncology
2024, Vol 34, Num 3 Page(s): 083-088
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Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey
Huseyin TOKGOZ1, Umran CALISKAN1
Necmettin Erbakan University, Meram Faculty of Medicine, Department of Pediatric Haematology, Konya, TURKEY
Keywords: Langerhans cell histiocytosis, Child
The aim of the study is to present the experience of children diagnosed with Langerhans cell histiocytosis (LCH) at our center. The medical records of the children with LCH were screened in terms of gender, age at diagnosis, clinical findings, risk groups, treatment modalities and outcome. The study included 9 patients with a median age of 22 months. The most common clinical finding is bone lesions (%88,8), followed by hepatosplenomegaly (%44.4), lymphadenopathy (%44.4), seborrheic dermatitis (33,3%) and jaundice (33.3%). Of the 9 patients, 3 (33.3%) had single system disease [1 (11.1%) had unifocal disease, 2 (22.2%) had multifocal disease], 6 (66.6%) had multisystem disease [2 (22.2%) had risk organ negative and 4 (44.4%) had risk organ positive]. At the end of the 6th week of therapy, 5 patients (55.5%) had a better response, 1 (11.1%) had an intermediate response and 3 (33.3%) had a worse response. In term of outcome, 5 (55%) patients were in complete remission, 3 (33%) patients were died, and 1 patient was lost to follow up. All of died children were in multisystem disease with risk organ involvement and worse responder at the 6th week of therapy. In children with LCH, most important factors determining the prognosis are risk organ involvement, multisystem disease and response at 6th week of treatment. However, new treatment modalities such as new drugs or stem cell transplantation are required for children with risk organ positive multisystem disease, as the prognosis remains poor.
Huseyin TOKGOZ1, Umran CALISKAN1
Necmettin Erbakan University, Meram Faculty of Medicine, Department of Pediatric Haematology, Konya, TURKEY
Keywords: Langerhans cell histiocytosis, Child
The aim of the study is to present the experience of children diagnosed with Langerhans cell histiocytosis (LCH) at our center. The medical records of the children with LCH were screened in terms of gender, age at diagnosis, clinical findings, risk groups, treatment modalities and outcome. The study included 9 patients with a median age of 22 months. The most common clinical finding is bone lesions (%88,8), followed by hepatosplenomegaly (%44.4), lymphadenopathy (%44.4), seborrheic dermatitis (33,3%) and jaundice (33.3%). Of the 9 patients, 3 (33.3%) had single system disease [1 (11.1%) had unifocal disease, 2 (22.2%) had multifocal disease], 6 (66.6%) had multisystem disease [2 (22.2%) had risk organ negative and 4 (44.4%) had risk organ positive]. At the end of the 6th week of therapy, 5 patients (55.5%) had a better response, 1 (11.1%) had an intermediate response and 3 (33.3%) had a worse response. In term of outcome, 5 (55%) patients were in complete remission, 3 (33%) patients were died, and 1 patient was lost to follow up. All of died children were in multisystem disease with risk organ involvement and worse responder at the 6th week of therapy. In children with LCH, most important factors determining the prognosis are risk organ involvement, multisystem disease and response at 6th week of treatment. However, new treatment modalities such as new drugs or stem cell transplantation are required for children with risk organ positive multisystem disease, as the prognosis remains poor.
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