RT Book, Section
A1 Smith, Graeme
SR Print(0)
ID 1152482488
T1 Low-risk myelodysplastic syndrome
T2 Problem Solving in Haematology
YR 2018
FD 2018
PB Clinical Publishing Oxford
PP New York, NY
SN 9781846920059
LK hemonc.mhmedical.com/content.aspx?aid=1152482488
RD 2021/03/08
AB A  70-year-old  otherwise  fit  and  healthy  female  had  been  diagnosed  3  years  previously  with  myelodysplastic  syndrome  (MDS;  French–American–British  [FAB]  classification  subtype  refractory  anaemia).  Cytogenetic  analysis  was  not  done,  as  it  was  considered  unnecessary  in  this  age  group.  The  patient  had  a  haemoglobin  concentration  of  7.5  g/dl,  white  cell  count  4  ×  109/l,  neutrophils  2.4  ×  109/l  and  platelets  450  ×  109/l  at  diagnosis.  Red  cell-transfusion  therapy  was  commenced,  with  a  transfusion  interval  of  3  weekly,  to  temporarily  relieve  symptoms  of  anaemia.  A  trial  of  recombinant  human  erythropoietin  therapy  with  30  000  units  per  week  for  6  weeks,  escalating  to  60  000  units  per  week  for  6  weeks,  produced  no  erythroid  response.  Serum  ferritin  assayed  after  3  years  of  red  cell-transfusion  dependence  was  4500  mg/l.  A  bone  marrow  examination  was  repeated  to  confirm  disease  stability  with  a  view  to  commencing  iron-chelation  therapy.  Serum  erythropoietin  concentration  was  2000  IU/l.  The  morphology  confirmed  World  Health  Organization  (WHO)  classification  subtype  refractory  cytopenia  with  multilineage  dysplasia,  which  was  changed  to  a  diagnosis  of  5q-  syndrome  when  cytogenetic  analysis  revealed  all  20  metaphases  showing  del(5)(q13-33)  as  the  sole  abnormality.Should  a  trial  of  recombinant  erythropoietin  have  been  offered?Is  there  a  therapeutic  option  to  modify  disease  and  promote  erythropoiesis?