Case: A boy of 4 yrs with Langerhans Cell Histocytosis

Mushfiq, a boy of 4 yrs presented with fever for 7 days, gradual pallor for 2 months, and multiple swelling over the scalp for 1 month.

On admission, he was febrile, toxic and pale. He was undernourished, his pulse was 110/m,BP -105/65 mmHg,respiration 25/m, temp-103 degree F, anaemia +++, jaundice-absent,no bleeding tendency, no lymphadenopathy, no bony tenderness and no hepatosplenomegaly.

The swelling over the skull are multiple,firm, non tender and fixed with the underlying structure.

on investigation, Hb-4.5 gm/dl, RBC-2.06m/cmm, MCV-85.4 fl, MCH 21.8 pg, MCHC 25.6 g/dl, WBC-7500/cmm, Neutrophil-60%,Lymphocytes -32%,Monocytes-04%,Eosinophil-04%. PBF shows anisosytosis and anisocromia with roulex formation, WBC mature with a few myelocytes, platelet suggestive of chronic disorder/infection.



X-ray of the skull shows focal scalp swelling in frontal and parietal region, pepper-potlucencies in the vault and subtle erosion on the outer tables of vault underneath the swelling and sutural diastasis suggestive of Langerhans cell histocytosis with raised intracranial pressure.

FNAC from the scalp growth shows hypercellualr material containing small round to oval cells having lobulated and folded nuclear with opened up chromatin;these cells are arranged diffusely and the cells show mild cellular atypia; the backgrond shows necrotic tissue debris suggestive of 'small round cell tumour' where Langerhans cell histocytosis may be considered.

CT scan of the brain shows extensive intracranial hyperdense massesshowing distribution along dura and frontal parenchyma. No mass effect or midline shift is present; there are multiple scalp swelling , some of them show underlying outer table erosion; there is splitting of sagital and coronal suture. The impression is of Langerhans cell histocytosis.

The patient was refered to special cancer center for further management.


Dr. Badrud Doza
Dr. Harun

Link:
Langerhan's Cell Histocytosis- Boston Children Hospital
Langerhan's Cell Histocytosis-Medscape.com