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 1189334756
T1 Primary Myelofibrosis
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=1189334756
RD 2024/04/19
AB Primary  myelofibrosis  is  a  chronic  myeloid  neoplasm  that  originates  in  mutations  in  a  multipotential  hematopoietic  cell,  possibly  the  lymphohematopoietic  stem  cell.  The  disease  is  characterized  by  (1)  anemia;  (2)  splenomegaly;  (3)  increased  CD34+  cells,  immature  granulocytes,  erythroid  precursors,  and  teardrop-shaped  red  cells  in  the  blood;  (4)  increased  dysmorphic  megakaryocytes,  the  cytokines  from  which  induce  marrow  fibrosis;  and  (5)  osteosclerosis.The  designation  (name)  for  this  clonal  neoplasm  has  been  problematic  throughout  the  years.  The  decision  to  call  it  primary  myelofibrosis  is  a  failure  to  understand  its  pathobiology.  There  are  no  tumors  of  connective  tissue  fibers.  The  disease  is  principally  a  profound,  neoplastic  expression  of  exaggerated  and  profoundly  disordered  hematopoiesis,  with  disordered  megakaryocytopoiesis  being  the  most  prominent  and  consistent  finding  and  the  basis  for  the  fibrosis  characteristic  of  the  disease.  The  primary  abnormality  of  megakaryocytopoiesis  is  amplified  by  the  fact  that  CD34+  cells  from  patients  with  this  disease,  when  grown  in  culture,  generate  24-fold  the  clonal  megakaryocytes  as  the  number  of  megakaryocytes  generated  by  normal  CD34+  cells.  Thus,  this  disease  most  closely  conforms  to  chronic  megakaryocytic  leukemia,  using  the  standard  convention  of  naming  a  myeloid  neoplasm  by  its  dominant  morphologic  expression  (eg,  monocytic  leukemia,  erythroid  leukemia).