CENTRAL NERVOUS SYSTEM INVOLVEMENT IN LANGERHANS CELL HISTIOCYTOSIS: DIFFERENT SHADES OF THE DISEASE
İnternational Society of pediatric hematologic oncology, Athens, Yunanistan, 22 - 24 Ekim 2023, cilt.70, ss.33, (Özet Bildiri)
- Yayın Türü: Bildiri / Özet Bildiri
- Cilt numarası: 70
- Doi Numarası: 10.1002/pbc.30714
- Basıldığı Şehir: Athens
- Basıldığı Ülke: Yunanistan
- Sayfa Sayıları: ss.33
- İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet
Özet
Central nervous system involvement in Langerhans Cell Histiocytosis (CNS-LCH) can occur as isolated LCH of the brain, in the setting of MS-LCH or in the form of neurodegeneration. As the disease has a wide clinical and radiological heterogeneity, diagnosis and treatment is challenging. Herein, 5 children with CNS LCH are presented. Since 2018, 4 girls,1 boy (age range 10-14 years) were diagnosed with CNS - LCH other than isolated pituitary stalk involvement. Three children had previous diagnoses of LCH in the form of Pulmonary involvement (#1), Multisystem Disease (#2) and CNS-Risk bone disease (#5). Patients #2, #3 and #5 had Diabetes Insipidus on admission. Headache and cognitive deterioration were the main complaints of 4/5 patients and gait, and speech disturbances were the presenting symptoms in one. Two children were diagnosed 2 and 4 years after the onset of symptoms, one child had CNS involvement 5 year after the primary diagnosis. Radiologically typical imaging findings in posterior fossa structures were noted in all. In one patient choroid plexus involvement was present. Based on the clinical and radiological findings, 2 children received IVIG, 1 child received systemic chemotherapy, 1 child received systemic chemotherapy and targeted therapy. Patient #5 was given supportive treatment due to familial disproval of any systemic therapy. Treatment resulted in slight clinical improvement on neurological scales with stable radiological findings in 2 patients, stable clinical and radiological disease in one patient. In patient #5 both clinical and radiological improvement were noted on follow-up. Due to its rarity and low level of awareness, the time to diagnosis of CNS-LCH was long in 3 of our patients with more pronounced clinical and radiological presentations. A standard algorithm for surveillance and diagnosis of CNS involvement is necessary in children with LCH