RT Book, Section
A1 Beer, Philip A.
A1 Green, Anthony R.
A2 Kaushansky, Kenneth
A2 Lichtman, Marshall A.
A2 Prchal, Josef T.
A2 Levi, Marcel M.
A2 Press, Oliver W.
A2 Burns, Linda J.
A2 Caligiuri, Michael
SR Print(0)
ID 1121098245
T1 Essential Thrombocythemia
T2 Williams Hematology, 9e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071833004
LK accessmedicine.mhmedical.com/content.aspx?aid=1121098245
RD 2024/04/18
AB SUMMARYEssential  thrombocythemia  is  a  clonal  stem  cell  disorder  characterized  by  an  overproduction  of  platelets  and  associated  with  mutations  in  the  JAK2,  CALR,  or  MPL  gene.  Complications  include  thrombosis  (predominantly  arterial),  hemorrhage,  and  progression  to  myelofibrosis  or  acute  myeloid  leukemia.  Diagnosis  requires  exclusion  of  reactive  thrombocytosis  and  other  myeloid  malignancies  associated  with  a  raised  platelet  count.  Therapy  is  aimed  at  reducing  thrombotic  complications  and  includes  modification  of  known  cardiovascular  risk  factors  and  antiplatelet  therapy  for  the  majority  of  patients.  Those  at  high  risk  of  thrombosis  are  also  considered  for  cytoreductive  therapy  with  agents  such  as  hydroxyurea,  anagrelide  or  interferon-α.  Although  the  majority  of  patients  can  expect  to  live  for  many  years,  mortality  rates  are  increased  compared  to  the  general  population  as  a  consequence  of  disease  complications.