Langerhan Cell Histiocytosis Flashcards
1
Q
Sites of LCH involvement
A
Everywhere has been described except GU and kidney
Bone ( most common) Skin CNS- pituitary, CNS disease or Neurodegenerative Lungs Thymus Thyroid GI Liver- can progress to PSC Spleen Bone marrow Lymph nodes
2
Q
Classification of LCH based on sites
A
Single system LCH
Univocal or multifocal
Multisystem LCH
With or without risk organ involvement
Risk organs are:
Liver, spleen, bone marrow
NOT lung anymore in pediatric patients
3
Q
Work up/ staging for LCH
A
CBC Renal labs, liver enzymes and coagulation profile Electrolytes First am U/A and specific gravity CXR Abdo U/S Skeletal survey incl skull Bone scan
Optional depending on symptoms
CT head if mass on skull
May need to do a water deprivation to diagnose DI
MRI or signs of DI
Bone marrow biopsy with BRAF PCR it cytopenias
Liver biopsy if unexplained hypoalbuminemia
