RT Book, Section
A1 Young, Neal S.
A2 Kaushansky, Kenneth
A2 Prchal, Josef T.
A2 Burns, Linda J.
A2 Lichtman, Marshall A.
A2 Levi, Marcel
A2 Linch, David C.
SR Print(0)
ID 1180479837
T1 Pure Red Cell Aplasia
T2 Williams Hematology, 10e
YR 2021
FD 2021
PB McGraw-Hill Education
PP New York, NY
SN 9781260464122
LK accessmedicine.mhmedical.com/content.aspx?aid=1180479837
RD 2024/04/19
AB SUMMARYPure  red  cell  aplasia  is  defined  by  the  combination  of  anemia,  reticulocytopenia,  and  the  absence  of  erythroid  precursors  on  the  marrow  examination,  but  the  pathophysiologies  are  diverse.  In  children,  a  genetic  defect  in  ribosome  protein  genes  is  responsible  for  Diamond-Blackfan  anemia.  In  older  adults,  pure  red  cell  aplasia  is  usually  immune-mediated,  sometimes  associated  with  thymoma,  and  responsive  to  immunosuppressive  therapies.  The  syndrome  can  overlap  with  other  diseases,  especially  large  granular  lymphocytosis,  or  complicate  them,  as  with  chronic  lymphocytic  leukemia  or  myelodysplastic  syndrome.  Parvovirus  B19  is  an  infectious  etiology,  acutely  producing  transient  aplastic  crisis  of  hemolytic  anemia  and,  when  persistent,  identical  to  pure  red  cell  aplasia.  The  exclusive  loss  of  erythrocyte  production  is  a  rare  complication  of  medical  drug  therapy,  but  pure  red  cell  aplasia  can  be  iatrogenic,  as  with  anti-erythropoietin  antibodies  after  administration  of  the  hormone  and  in  ABO-incompatible  allogeneic  transplantation  caused  by  persistence  of  host  isoagglutinins.