Myelodysplastic Syndromes (MDS) Diagnosis and Management: Dairion Gatot, Savita Handayani

MYELODYSPLASTIC SYNDROMES (MDS)
DIAGNOSIS AND MANAGEMENT
Dairion Gatot, Savita Handayani
Hematology-Oncology Medical Division

Dept of Internal Medicine
School of Medicine, Sumatera Utara University
Medan 2013
3
4
5
6
Work-up
Leukocyte:
ANC<2500/mm3 → 50%
ANC<1500/mm3 → 20-35%
1.CBC with differential ↑ WBC → rare (except patients with CML)
Hb<10 g/dL → 80%
Platelets → Thrombocytopenia → 25-50%
Peripheral blood smear abnormalities are a
hallmark of MDS
1.Hypogranulated neutrophils with abnormal
nuclei
2.Peripheral blood smear 2.Giant platelet
3.Polychromasia
4.Macrocytosis and anisopoikilocytosis
5.Reticulocytosis (rare)
Work-up
3.Bone marrow biopsy  Marrow cellularity → normal or ↑.

& aspiration with iron
stains
4.Cytogenetic analysis  Clonal cytogenetic abnormalities →

of bone marrow cells 30-79% of patients at presentation.
6.Serology HIV testing → if indicated.
7.Chemistry Folat, serum B12, serum iron/TIBC/ferritin,

serum erythropoietin level → before RBC
transfusion.
MDS Classification
 1. French America Brithish (FAB)

 2. World Health Organization (WHO)
 3. International Prognostic Scoring System (IPSS)
Bernett J et al Haemato 1982; 51; 189-199

Harris N et al Ann Oncol 1999; 10;1419-1432
Greenberg P at al Blood.1997;89;2079-2088
FAB classification of MDS
FAB type % blasts in % blasts in % ringed Blood
blood marrow sideroblasts monocytes
Refractory
anemia <1 <5 <15 Rare
Refractory
anemia with
<1 <5 >15 Rare
ringed
sideroblast
Refractory
anemia with <5 5-20 <15 Rare
excess blasts
Refractory
anemia with
>5 21-30 <15 variable
excess blasts in
transformation
Chronic
myelomonocytic <5 1-20 <15 >109/L
leukemia
World Health Organization classification of MDS
WHO type Blood findings Marrow findings
Refractory anemia (RA) Anemia Only erythroid dysplasia
No or rare blasts <5% blasts
No Auer rods <15% ringed sideroblasts
No Auer rods
Refractory anemia with ringed sideroblasts Anemia Same as RA except > 15%
No blasts ringed sideroblasts
No Auer rods
Refractoey cytopenia with multilineage dysplasia 2-3 cytopenias Same as RA except dysplasia
(RCMD) No or rare blasts in > 10% of cells in > 2 lines
No Auer rods
<1,000 monocytes/L
Refractory cytopenia with multilineage dysplasia and Same as RCMD Same as RCMD except > 15%
ringed sideroblasts ringed sideroblasts
Refractory anemia with excess blasts-1 Cytopenias Uni or multilineage dysplasia
<5% blasts 5-9% blasts
No Auer rods No Auer rods
<1,000 monocytes/ L
Refractory anemia with excess blasts-2 Cytopenias Uni or multilineage dysplasia
5-19% blasts 10-19% blasts
Auer rods + Auer rods
Unclasisified MDS Same as RCMD Unilineage dysplasia
<5% blasts
MDS associated with isolated del(5q) Anemia Normal to increased dysplastic
Platelet normal or megakaryocytes
increased <5% blasts
<5% blasts No Auer rods
International prognostic scoring system for MDS
Prognostic factor Points
Percentage of blasts
<5% 0
5-10% 0.5
11-20% 1.5
21-30% 2
Cytogenetic features
Normal karyotypes, -Y,5q-,20q- 0
Other 0.5
Abnormal chromosome 7,> 3 abnormalities 1
Cytopenia*
0-1 type 0
2-3 types 0.5
MDS Classifications System IPSS
IPSS Risk Group Total % Lifetime Median Years Median

Score AML evolution to AML Survival (yr)
Low 0 19 9.4 5.7

Intermediate-1 0.5-1 30 3.3 3.5
Intermediate-2 1.5-2 33 1.1 1.2
High >2 45 0.2 0.4
Faderl et al.in DeVita VT Jr,Hellman S, Rosenberg SA,eds.Cancer: Principles &Practice of Oncology.2005:2144-2154

List AF et al, Haematology (am.Soc.Haematol Educ Program).2004:297-317
Treatment
 Supportive Care
 Observation → low risk disease who do not require
transfusions
 Transfusions
o transfusions of erythrocytes & platelets → to
maintain an adequate quality of life & to avoid
complications of severe anemia and
thrombocytopenia.
o chronic transfusions → iron overload → iron
chelation with deferoxamine may be necessary.
Treatment
 Growth factors
 Erythropoietin
• Efficacy → ↑ Hb & ↓ transfusion requirements.
• Formulations : epoeitin alfa (Procit®) &
darbepoietin alfa (Aranesp®) .
• Epoeitin alfa dosing options :
– 150-300 U/kg SC daily.
– 20,000 U SC three times weekly.
– 40,000 U SC weekly→ the effect of this
schedule is not known.
Treatment
 Filgrastim (G-CSF) and sargramostim (GM-CSF)
• Efficacy → ↑ neutrophil count & ↓ the risk of
infectious complications.
• In conjunction with epoeitin alfa → ↑ the erythroid
response rate ~ 40%.
• Doses → 75-100g/day.
• Indications (debatable) → Anemia not responding to
epoeitin alfa; Neutrophenic patients with infections;
and ANC < 200/L
 Chelating agents → for transfussion-induced iron
overload.
 Vitamin therapy → ineffective hematopoiesis may
deplete vitamin B6, B12, and folate.
Treatment
 First drugs for MDS became available only recently

• VIDAZA (azacytidine) → US FDA approval in May
2004
• REVLIMID (lenadinomide)→US FDA approvel in
Dec 2005 for low risk or int-1 with 5q
• DACOGEN (dectabine) → US FDA approval in
may 2006
 Transplantation → the only known curative treatment.
CONCLUSION
 MDS → a heterogenous group of clonal bone marrow
disorders → ineffective hematopoiesis and a variable risk
of transformation to acute myeloid leukemia.
 MDS classification → FAB, WHO & IPSS.
 Treatment :
• Supportive → observation, transfusion, growth factor
(erythropoietin, filgrastim, sargramostim), chelating
agents and vitamin therpay.
• First drug → Vidaza, Revlimid, Dacogen.
• Transplantation → curative treatment.

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MYELODYSPLASTIC SYNDROMES (MDS)

DIAGNOSIS AND MANAGEMENT

Dairion Gatot, Savita Handayani

Hematology-Oncology Medical Division

3.Bone marrow biopsy  Marrow cellularity → normal or ↑.

4.Cytogenetic analysis  Clonal cytogenetic abnormalities →

7.Chemistry Folat, serum B12, serum iron/TIBC/ferritin,

 1. French America Brithish (FAB)

Bernett J et al Haemato 1982; 51; 189-199

Prognostic factor Points

IPSS Risk Group Total % Lifetime Median Years Median

Low 0 19 9.4 5.7

Faderl et al.in DeVita VT Jr,Hellman S, Rosenberg SA,eds.Cancer: Principles &Practice of Oncology.2005:2144-2154

 First drugs for MDS became available only recently

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