PATHOLOGICAL CHANGES
Lung Tissue from:
1) open lung biopsy
2) Bronchoscopy with BAL Electron Microscopy is essential for diagnosis. X bodies with birbeck granules seen and confirmatory. Micro nodules or diffuse infiltrates noted. It is a proliferative disorder. LCH and Smoking: In adults, heavy smokers have a higher incidence of LCH. Tobacco is thought to act via glycoprotein as an immunostimulant. Cytokine IL-2 and lymphocytes are less proliferative with remission demonstrated in an adult treated with IL-2. Neonatal LCH: Many typical skin rashes are noted with LCH that are easily misdiagnosed. Skin lesions include: 1) Vecsicopustular(+/- crusting) 2) Eczema (seborrhea - like ) dermatitis
3) Mucosal lesions (erosions, granulomas, petechiae)
4) Erythema papules
5) Nodular ulceration lesions
6) Petechiae
CLICK ON ANY IMAGE BELOW TO SEE AN ENLARGEMENT.
Cutis aplasia was a consideration in this case initially.
Neonatal
forms are another classification again including:
1) Congenital self healing reticulocytois (CSHRH)
2) Solitary congenital indeterminate histiocytosis (ICH)
3) Generalized eruptive histiocytosis (GEH)
TREATMENT
Variable
depending on presention and organs involved. Watchful waiting to steroid
treatment +/- alkylating agents like Vinblastine, and Mecoptopurine(6MP).
A pediatric oncologist should be involved in the chemotherapeutic regime
as protocols now exist for chemo.
SUMMARY
In
summary pediatric histiocytosis can present with severe lung manifestations
despite little clinical chest manifestations until extensive disease
has occurred. Smoking is a known risk factor in adults, and should be
strongly discouraged at all clinical follow-ups. Neonatal HCT is not
a benign disease and can reoccur in other organs, so long term follow
up is suggested. Long-term follow-up is recommended with PFT's, once
children are old enough to perform these tests. Baseline CXR should
be done on all LCH cases to document presence or absence of lung disease.
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