Case report – HLH

Introduction
Case history
One year and two-month old child was admitted to general pediatric ward due to right leg
cellulitis.
He was 2nd child of the non-consanguineous parents. He was apparently well up to 6months of
age. Mother noticed hypo pigmented rashes in forehead then in back.it was non itchy, but spread
to back from face. honey comb scaly rash was appeared in scalp at 9moth of age. mother seek
medical treatment but there was no response. Around 11month of age, painless small lump was
noted on scalp just above right ear. It was increased slowly. Medical treatment was obtained
twice but there was no response to treatment. he had no history of trauma.
This is 2nd pregnancy. antenatal and perinatal histories were uneventful. Birth weight was 2.7 kg.
weight is on less than -3SD.OFC is on -1SD to median. On examination baby have healed
seborrheic dermatitis in scalp and varying size of hypo pigmented patches in forehead and back.
there is 2cm * 0.5cm surgical scar just above the right ear without bony defect. There is no
lymphadenopathy. There is no nappy dermatitis. Liver is just palpable and spleen is 3cm. there
were right sole cellulitis .
Full blood count showed total count of 13280 and N-36% and L-55%. His hemoglobin is
10.4g/dl. Platelet count was normal. ESR was 50mm/1st H and CRP was negative. Liver. renal
functions were normal. Serum electrolytes includes calcium were normal. initial ultra sound scan
of abdomen was normal. ultra sound scans of neck showed normal thyroid and right side reactive
lymphadenopathy. All long bones , lateral skull Xray were normal.
Contrast enhanced computed chromatography (CECT) abdomen ,chest and brain showed lytic
expansion lesion of left posterior chest wall with associated soft tissue mass , normal bilateral
lung fields , L1 vertebral bodies complete destruction with pathological fractures and associated
soft tissue mass , no supra renal mass , bilateral normal kidneys , normal liver, there is contrast
enhanced mass lesion in right temporal region of skull measured 2.9cm * 2cm with erosion of
temporal bone .lesion was communicated with Dural covering of brain. Multiple lytic lesion in
ribs, spine and right side of face were found. Punch Skin biopsy from scalp revealed
acanthocytes epidermis with scab formation there are numerous histiocytes in upper dermis with
mitosis with few erosion. Temporal scalp lump excision biopsy revealed immunomorphological
features were compatiple with Langerhans cell histiocytosis (immunohistochemistry CD1a –
diffuse membranous positivityin Langerhans cells, S100 – strong and diffuse cytoplasmic and
nuclear positivity .NSE – no strong positivity, Ki 67 proliferartion index -24%)
Langerhans cell histiocytosis and made baby underwent chemotherapy.