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Histiocytic syndromes in children
N Cetingül1, S Oztop, K Kavakli
1Department of Pediatrics and Pathology, Ege University Faculty of Medicine, Izmir.
The Turkish Journal of Pediatrics
|July 1, 1997
Summary
Childhood histiocytosis, primarily Langerhans cell histiocytosis (LCH), often presents with skeletal issues. Effective treatments exist for localized LCH, but disseminated forms and other histiocytic disorders carry significant risks.
Area of Science:
- Pediatric Hematology and Oncology
- Immunology
- Cell Biology
Background:
- Histiocytoses encompass proliferative disorders of the mononuclear phagocyte system with largely unknown causes.
- Childhood histiocytoses frequently involve excessive Langerhans cells, termed Langerhans cell histiocytosis (LCH).
Purpose of the Study:
- To analyze the clinical characteristics, manifestations, and treatment outcomes of pediatric histiocytosis patients.
- To identify prognostic factors influencing disease progression and survival in histiocytosis.
Main Methods:
- Retrospective review of 15 pediatric patients diagnosed with histiocytosis syndrome.
- Evaluation of treatment responses to chemotherapy, radiotherapy, and prednisone.
Main Results:
- Langerhans cell histiocytosis (LCH) was the predominant diagnosis, with skeletal involvement being the most common manifestation.
- Patients with unifocal and multifocal LCH showed good responses to radiotherapy and chemotherapy.
- Two patients with disseminated LCH and one with malignant histiocytosis died due to progressive disease or relapse.
- A partial response to prednisone was observed in a patient with Rosal-Dorfman disease.
Conclusions:
- Langerhans cell histiocytosis is the most frequent type of childhood histiocytosis, often affecting the skeleton.
- Radiotherapy and chemotherapy are effective for localized LCH, while disseminated disease and other histiocytic types have poorer prognoses.
- Organ dysfunction and patient age are critical factors impacting the outcome of histiocytosis syndrome.