Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
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 |
