RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164076789
T1 Letterer-Siwe Disease (LSD)
T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9781259861789
LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164076789
RD 2024/04/19
AB It  is  a  childhood  immunologic  disorder  characterized  by  pathologic  proliferation  of  histiocytes  caused  by  the  Langerhans  cells.  It  is  one  of  the  four  recognized  clinical  syndromes  of  Langerhans  cell  histiocytosis  (LCH).  It  causes  approximately  10%  of  LCH  disease  and  is  the  most  severe  form.  It  is  characterized  by  skin  lesions  (scaly  skin  involving  the  scalp,  ear  canals,  and  abdomen),  lymphadenopathy,  osteolytic  lesions,  and  hepatosplenomegaly.  Other  organs  involved  are  the  spleen,  brain,  lungs,  and  bones.  The  presence  of  diabetes  insipidus  (DI)  must  be  considered.  Presents  nonmalignant  growths  that  represent  accumulation  of  histiocytes.  It  is  associated  with  a  poor  prognosis  (70%  mortality).