Dr. Atul A. Kulkarni, M.D.(Paed)*, Dr. Kiran B. Masal, Resident **
Department of Paediatrics, Ashwini Sahakari Rugnalaya & Research Centre, Solapur. *, Department of Paediatrics, Ashwini Sahakari Rugnalaya & Research Centre, Solapur. **
Case History: 7 years female child presented with Polyuria, polydipsia, headache (mainly in occipital region) since 2 months. Drowsiness since 8 days, patient came in altered sensorium.
  • Hb - 7.5, TLC - 4800, Platelets - 1,50,000/μl
  • CSF/BSL/ Urine examamination - normal
  • Serum electrolytes: Na -192, K - 4.5, CI - 102 suggestive of diabetes insipidus

X-ray skull-Osteolytic lesion in occipital region CT head - Pituitary adenoma Biopsy - Type 1 histiocytosis.

Childhood histiocytosis constitutes a diverse group of disorders. Disease Cellular characteristics: Class 1 Langerhans Cell Histiocytosis, Langerhans Cells (CDI Positive) with Birbeck granules.
Clinical manifestations
  • Skeleton involvement in 80% cases. Osteolytic lesions - single or multiple mostly in skull.
  • Seborrheic dermatitis, Hepatosplenomegaly, Lymphadenopathy (localized or disseminated) - Hematological disorders: Anemia, Leucopenia, Thrombocytopenia
  • Pulmonary infiltration with respiratory distress and coughing
  • Growth retardation (Pituitary or Hypothalamic dysfunction)
  • Systemic manifestation in severely affected patient

Single system disease - Curettage or low dose local radiation therapy. Multisystem disease - Systemic multiagent chemotherapy (vinblastine or etoposide)
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