Saima Aamir, Anwarul Haque.
Langerhan Cell Histiocytosis - A case report.
Int J Pathol Jan ;3(1):48-51.

A 9 months old boy presented with fever and pallor for 15 days, progressively increasing abdominal distension for 1 month and rash on scalp and abdomen since 5 months. There was history of fits at the age of 5 months. He was a product of non-consanguineous marriage. Rest of the siblings were alive and healthy. On examination he was a sick looking pale child with maculopapular rash on abdomen, soles, palms, scalp, postauricular area, cheek and concha with sparing of chest, limbs and back. There was hepatosplenomegaly. The differential diagnosis included anemia, seborrhic dermatitis, storage disease or infectious disease. Laboratory investigations revealed pancytopenia with Hb count of 1.9 gm/ dl, WBC count 2500/ mm3 and platelet count of 40000/mm3. Bone marrow aspiration and biopsy were performed which showed hypercellular marrow with erythroid hyperplasia and moderate degree of fibrosis. There was no evidence of lymphoma, leukemia or any abnormal cells in bone marrow. FNAC of liver and spleen were performed with 23 gauge needle using stab technique from 3 different areas. The smears were fixed with 95% ethanol and stained with hematoxylin and eosin. Skin biopsy specimen was fixed in 10% buffered formalin, routinely processed and embedded in paraffin. The sections were stained with hematoxylin and eosinA diagnosis of Langerhan cell Histiocytosis was made on FNAC of liver and spleen. Skin biopsy also revealed same picture. He was given injection Vincristine but unfortunately he succumbed to his disease.

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