Myelodysplastic Syndrome (MDS) Low Risk
Haematological Pathway
for
South Wales Cancer Network
Document Control Sheet
Organisation
Specialty/Project
Document Title
Document Number
Version
1.0
Approved by
South Wales
Haematology Cancer
Network Group
South Wales Cancer Network
Haematological Site Specific Group
Myelodysplastic Syndrome (MDS) Low Risk
Haematological Pathway
05/007
Author/s
Dr W Ingram
Dr J Kell
Approval date
Ratified by
8.1.14
8.1.15
Dr C Fegan
Date of next review
Low Risk Myelodysplastic Syndrome (IPSS Low/Int-1)
NSAG Patient Care Pathway
Demographics
- The overall incidence of MDS is 4 per 100,000 increasing to >30 per
100,000 in patients over 70 years old.
Diagnosis
- Full blood count with differential WBC including monocyte count
- Blood film analysis
- Reticulocyte count
- Bone Marrow aspirate and trephine biopsy
o Morphology
o Flow cytometry quantification of blast population
o Iron stain
o Reticulin stain
o Cytogenetic analysis
- Additional investigations:
o EPO level (if Hb <100 g/l)
o JAK2 status (in suspected cases of MDS/MPN overlap syndromes)
o PNH screen
o CMV, Hepatitis B/C, HIV
Staging and Prognostication
- WHO classification of MDS
- Both the IPSS and the WPSS should be documented at diagnosis.
IPSS-R can be used for prognostication, but not to guide therapy
- Additional investigations to aid prognostic stratification:
o Ferritin
Information Required at MDT
- The WHO classification should be documented at MDT.
- The IPSS and WPSS (If available the IPSS-R may be recorded)
- All essential diagnostic information and any relevant additional
investigations should be recorded.
Treatment
All new MDS cases should be discussed at an MDT.
All MDS patients should be evaluated for suitability for entry into
local/regional clinical trials.
Further support for patients can be accessed via the MDS Patient Forum
(www.mdspatientsupport.org.uk)
Supportive Care
- Supportive care remains the mainstay of treatment for the majority of
patients with Low/Int-1 risk MDS.
- Local guidelines should be followed with respect to antimicrobial
prophylaxis in neutropenic patients.
- Red cell transfusions for symptomatic anaemia.
- Platelet support for symptomatic thrombocytopenia.
- G-CSF may be considered for patients with low risk disease and
recurrent infections.
- ESAs and iron chelation are discussed in further detail below.
Myelodysplastic Syndrome (MDS) Low Risk
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Iron chelation
- The BCSH task force has taken the view that it cannot routinely
recommend iron chelation in MDS as only limited survival data has
been reported in retrospective studies.
- Entry into clinical trials is strongly encouraged.
- A pragmatic view has been taken to consider iron chelation for patients
with good prognosis MDS (RA/RARS/del(5q)) or those who may be
considered for allogeneic transplantation.
- Ferritin >1000 ng/l or >20 units transfused red cells with ongoing
transfusion need should trigger iron chelation therapy in suitable
patients.
- Desferrioxamine is first line therapy with Deferasirox recommended for
patients intolerant of desferrioxamine. Deferiprone could be considered
in patients with normal baseline neutrophil counts.
Erythropoietin Stimulating Agents (ESA)
- ESA therapy should be considered for anaemic MDS patients with IPSS
score of Low or Int-1 who fulfil the below criteria for predicting
response (Score 0 and 1).
- Non-sideroblastic phenotypes should start with EPO or DA alone. Start
EPO at 30,000 units per week for eight weeks, if no response increase
to 60,000 units per week or 30,000 units twice per week for a further
8 weeks. Alternatively start DA at 300ug once every 14 days or 150ug
once every 7 days, if no response after eight weeks increase to 300ug
per week for a further 8 weeks.
- For sideroblastic phenotypes (RARS and RCMD-RS) ESA should be
combined with G-CSF starting at 300ug per week in 2/3 divided doses
(or 105ug 2/3 times per week) increasing to 300ug three times per
week in non-responders. Aim for doubling of WBC if < 1.5 x 109/l or
WBC 6-10 x 109/l.
- The haemoglobin should not be allowed to rise above 12g/dl.
Predictive response
=7%
Transfusion need
<2 units
RBC/month
≥2 units
RBC/month
to ESA: Score 0 = 74%, Score 1 = 23%, Score 2
Point
0
S-EPO
<500 U/l
Point
0
1
≥500 U/l
1
Immunosuppressive Therapy
- ATG (horse) followed by ciclosporin may be considered in patients with
Low/INT-1 MDS who are less than 60 years of age and have a normal
karyotype or trisomy 8.
- Ciclosporin therapy alone may be considered for older patients
particularly in cases with hypocellular BM or associated autoimmune
phenomena.
MDS with del(5q)
- Supportive care including transfusions, ESAs and iron chelation are as
described above.
- Lenalidomide has recently been licensed within Europe and under
review by NICE. Access to this agent within Wales is currently only
available via the IPFR route whilst awaiting NICE review.
Myelodysplastic Syndrome (MDS) Low Risk
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-
Selected patients with del(5q) and IPSS Low/INT-1 may be candidates
for allogeneic stem cell transplantation. These include lenalidomide
treated patients who fail to achieve transfusion independence, those
losing their response, or patients with transfusion dependence not
considered suitable for lenalidomide.
Allogeneic Stem Cell Transplantation
- Allo-SCT is the only treatment with proven curative potential.
- All newly diagnosed MDS patients eligible for SCT should be referred
for discussion at the Myeloid MDT held at UHW.
Monitoring
- Monitoring should be tailored according to the patients’ symptoms and
degree of blood count abnormality.
- Ferritin should be monitored in patients potentially suitable for iron
chelation or allo-HSCT.
Discharge
- Patients with a confirmed diagnosis of MDS should remain under the
care of a haematologist.
Reference
BCSH Guideline: Guidelines for the Diagnosis and Management of Adult
Myelodysplastic Syndromes (Awaiting publication)
Z:\ststorage2\South Wales Cancer Network\SWCN 13\Tumour Site\Haematological\Pathways\MDS
Low Risk.doc
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