RT Book, Section
A1 Tharp, Michael D.
A2 Kang, Sewon
A2 Amagai, Masayuki
A2 Bruckner, Anna L.
A2 Enk, Alexander H.
A2 Margolis, David J.
A2 McMichael, Amy J.
A2 Orringer, Jeffrey S.
SR Print(0)
ID 1162814261
T1 Mastocytosis
T2 Fitzpatrick's Dermatology, 9e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9780071837798
LK dermatology.mhmedical.com/content.aspx?aid=1162814261
RD 2024/04/20
AB AT-A-GLANCEMASTOCYTOSISThe  hallmark  of  mastocytosis  is  a  pathologic  accumulation  of  mast  cells  in  tissues.Mastocytosis  occurs  at  any  age  but  children  are  more  commonly  affected.Cutaneous  findings  consist  of  hyperpigmented  macules,  papules,  or  nodules,  or  a  diffuse  infiltration  of  the  dermis.Most  children  only  have  skin  involvement,  whereas  adults  are  more  likely  to  have  systemic  disease.Mastocytosis  is  usually  associated  with  somatic  activating  mutations  of  c-kit  with  the  816  codon  mutation  being  most  common.Many  patients  have  few,  if  any,  symptoms,  but  some  experience  varying  degrees  of  flushing,  pruritus,  hypotension,  nausea,  dyspepsia,  and  diarrhea.Most  common  extracutaneous  tissues  involved  are  the  bone  marrow,  liver,  spleen,  and  lymph  nodes.Some  patients  may  develop  an  associated  myeloproliferative  or  myelodysplastic  disorder.No  effective  cure  currently  exists;  treatment  is  focused  on  controlling  symptomsMAST  CELLSMast  cells  are  derived  from  pluripotent  stem  cells.Stem  cell  factor  is  the  ligand  for  KIT  and  is  required  for  mast  cell  proliferation  and  survival.Mast  cells  release  both  preformed  and  newly  generated  mediators.