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15 Cards in this Set

  • Front
  • Back
what is the endocrine involvement with LCH
DI
growth failure
hypothyroidism
what interstitial pulmonary pattern is seen with LCH
honeycombing
clinical presentation of LCH
painful swelling of bones
persistent otitis/mastoiditis
mandible involvement ("floating teeth")
papular rash (can look like candida, can look like a viral rash)
HSM
LAD
radiographic bone findings in LCH
lytic skull lesions
collapse of vertebrae
presentation of neurologic syndromes in LCH
ataxia, dysarthria, dysmetria, behavior changes
findings on MRI in neurologic syndromes in LCH
masses or T2 hyperintense signal in cerebellar white matter, pons, or basal ganglia secondanry to neurodegeneration/gliosis
what constitutes high risk LCH
tx
involvment of liver, spleen, lung, BM
velban/pred/6MP +/- MTX
12 months
what constitues low risk LCH
tx
bone +/- skin, lymph nodes
velban/pred 6-12 months
cure rate in low risk LCH
virtually 100%
cure rate in high risk LCH
depends on response at 6 weeks of therapy

good response: 6% mortality
intermediate: 21%
nonresponder - 60%
what rheumatologic syndromes can be associated with HLH
kawasaki, JRA, lupus
CSF findings in CNS HLH
mononuclear pleocytosis
elevated protein
what are fibrinogen levels in HLH
low
what are the effects of defective NK and cytotoxic T cell function in HLH
unregulated cytokine production
no apoptosis of lymphs and monos
what genetic mutations in HLH can be tested?
Perforin 1 / FHL2 - PRF1 on 10q22
MUNC 13 homolog D / FHL3 - UNC13D on 17q25.1