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HISTIOCYTOSIS
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XVII Annual Conference of IAP Maharashtra State (Mahapedicon 2006, Solapur, 3-5th November 2006)
Dr. Atul A. Kulkarni, M.D.(Paed), Dr. Kiran B. Masal, Resident
Department of Paediatrics, Ashwini Sahakari Rugnalaya & Research Centre, Solapur.
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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.
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| Investigations : |
- Hb -7.5 TLC- 4800 Plat.-150000
- CSF/BSL/ Urine exam.- normal
- Serum electrolytes Na -192 K - 4.5 CI - 102 suggestive of diabetes insipidus.
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X-ray skull–Osteolytic lesion in occipital region CT head – Pituitary adenoma Biopsy – Type 1 histiocytosis.
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Histiocytosis:
Childhood histiocytosis constitutes a diverse group of disorder. Disease Cellular characteristics Class 1 Langerhans cell histiocytosis Langerhans cells (CDI Positive) with Birbeck granules
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| Clinical manifestations: |
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Skeleton involvement in 80% cases. Osteolytic lesions single or multiple mostly in skull.
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Seborrhic dermatitis, Hepatosplenomegaly, Lymphadenopathy (localized or disseminated) – Hematological disorder Anemia, Leucopenia, Thrombocytopenia
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Pulmonary infiltration with respiratory distress and coughing.
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Growth retardation (Pituitary or Hypothalamic dysfunction)
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Systemic manifestation in severely affected patient.
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| Treatment: |
| Single system disease - Curettage or low dose local radiation therapy. Multisystem disease - Systemic multiagent chemotherapy (vinblastine or etoposide) |
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| Last Updated on 01-04-2007
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