RT Book, Section
A1 Lichtman, Marshall A.
A1 Kaushansky, Kenneth
A1 Prchal, Josef T.
A1 Levi, Marcel M.
A1 Burns, Linda J.
A1 Linch, David C.
SR Print(0)
ID 1189334421
T1 Polycythemia Vera
T2 Williams Manual of Hematology, 10e
YR 2022
FD 2022
PB McGraw-Hill Education
PP New York, NY
SN 9781264269204
LK hemonc.mhmedical.com/content.aspx?aid=1189334421
RD 2024/04/18
AB Polycythemia  vera  (PV)  is  a  clonal  disorder  arising  from  somatic  mutations  of  a  hematopoietic  stem,  or  closely  related  multipotential  cell,  in  which  blood  cell  production,  notably  in  the  erythroid  lineage,  but  also  in  the  granulocytic,  monocytic,  and  platelet  lineages,  is  increased  and  is  independent  of  cytokine  regulation.  This  results  in  exaggerated  proliferation  and  accumulation  of  erythrocytic,  and  often  granulocytic  and  megakaryocytic,  precursors  in  the  marrow  and  their  mature  cells  in  the  blood.  PV  is  one  of  the  myeloproliferative  neoplasms  (MPNs),  along  with  essential  thrombocythemia  (ET),  primary  myelofibrosis  (MF),  and  chronic  myelogenous  leukemia  (CML).Three  MPNs  (PV,  ET  [Chap.  43],  and  MF  [Chap.  48])  may  share  a  common  molecular  abnormality/marker,  the  JAK2  kinase  V617F  mutation.  In  contrast,  CML  is  caused  by  a  different  molecular  alteration,  the  BCR/ABL  oncogene,  due  to  a  reciprocal  translocation  between  chromosome  9  and  chromosome  22,  t(9;22)(q34;q11),  and  the  production  of  the  BCR-ABL  protein  product  (Chap.  47).