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Myelodysplastic Syndrome

Myelodysplastic syndrome, or MDS, is a group of diseases in which the bone marrow doesn’t produce enough healthy blood cells. Red blood cells, white blood cells and platelets originate in the marrow and move into the bloodstream when they mature. In MDS, the marrow produces too many immature cells (blasts).

 

These blasts die in the marrow or soon after entering the bloodstream, resulting in too few healthy blood cells and low patient blood counts.

 

In its mildest form, myelodysplastic syndrome may be only anemia, low platelets or low white blood count, but higher-risk types can progress to acute myeloid leukemia (AML). Each year in Spain 3.000 new cases of MDS are diagnosed.

 

Ninety percent of myelodysplastic syndrome patients are over the age of 60, and 10-20% of diagnosed cases will progress to AML.

 

 

Types of Myelodysplastic Syndrome
 

There are many different types of myelodysplastic syndrome. Over the last 30 years, at least three classification systems have been created in an effort to categorize the various disease types. MD Anderson Madrid – Hospiten uses the International Prognostic Scoring System (IPSS), a numerical score to determine a patient's risk for myelodysplastic syndrome: 

 

Risk Level

IPSS Score

Low risk

0

Intermediate risk 1

0,5 – 1

Intermediate risk 2

1,5 - 2

High risk

≥2,5

 

The following factors are used to calculate the IPSS score: 

 

 

0,0

0,5

1,0

1,5

2,0

% Marrow Blasts

<5

5-10

 

11-20

21-30

Cytogenetics

Normal, -Y
del(5)q sola
del(20)q sola

Otros

-7, del(7)q
≥ 3 anormal

 

 

Cytopenias:

  • Hemoglobin (HGB) < 10 g/dl
  • Absolute Neutrophils (ANC) <1.500/ul
  • Platelets (PLT) <100K/ul

0/1

2/3

 

 

 

 

The IPSS score is also used by oncologists to predict age-related survival rates and progression to acute myelogenous leukemia in MDS patients.

 

 

No. of Patients

Low

Int-1

Int-2

High

A. Median Survival (yr)

Total Patients (%)

816

267 (33)

314 (88)

176 (22)

59 (7)

Median Survival (yr)

 

5,7

3,5

1,2

0,4

Age (yr)

 

 

 

 

 

<60

205 (25)

11,8

5,2

1,8

0,3

>60

611

4,8

2,7

1,1

0,5

<70

445 (54)

9,0

4,4

1,3

0,4

>70

271

3,9

2,4

1,2

0,4

B. 25% AML Evolution (yr)

Total Patients (%)

759

235 (51)

295 (39)

171 (22)

58 (8)

Median Survival (yr)

 

9,4

3,3

1,1

0,2

Age (yr)

 

 

 

 

 

<60

187 (25)

> 9,4 (NA)

6,9

0,7

0,2

>60

572

9,4

2,7

1,3

0,2

<70

414 (55)

> 9,4 (NA)

5,5

1,0

0,2

>70

345

> 5,8 (NA)

2,2

1,4

0,4

 

NR = not reached

While recognizing the usefulness of the IPSS, we developed a new prognostic model specifically for the Low and Int-1 subset of MDS patients, below. This is based on an analysis published in Leukemia, 2008; 22: 538-543.

 

Adverse Factor

Assigned
Score

Unfavorable cytogenetics

1

Age > 60 years

2

Hgb <10 g/dl

1

Plt <50 K/ul

2

Plt 50–200 K/ul

1

BM blasts >4%

1

In this analysis, diploid and 5q only were favorable cytogenetic, all others were considered as unfavorable cytogenetics.

Abbreviations: BM, bone marrow; Hgb, hemoglobin; Plt, platelets.

 

Estimated survival outcomes within each score range and proposed risk categories

 

Score

No. of patients

Median
(month)

Four-year survival (%)

Category

0

11

NA

78

1

1

58

83

 82

1

2

113

51

51

1

3

185

36

40

2

4

223

22

 27

2

5

166

14

9

3

6

86

16

7

3

7

13

9

NE

3
Abbreviations: NA, not assessable; NR, not reached.

There are multiple symptoms of myelodysplastic syndrome, many of them non-specific. Signs may include:

  • Easy bruising
  • Fatigue
  • Weight loss
  • Petechiae (tiny red spots just under the skin)
  • Fever
  • Frequent infections
  • Weakness
  • Shortness of breath

Having one or more of the symptoms listed above does not necessarily mean you have myelodysplastic syndrome However, it is important to discuss any symptoms with your doctor, since they may indicate other health problems.

News
Events

At this moment there are no events of Myelodysplastic Syndrome

Teaching

At the moment there are no courses of Myelodysplastic Syndrome

Clinical trials
Sym015(Anti-MET) en Pacientes con tumor solido avanzado.
Ensayo de fase 1a/2a, abierto y multicéntrico, para investigar la seguridad, tolerabilidad y actividad antitumoral de dosis repetidas de Sym015, una mezcla de anticuerpos monoclonales dirigida frente al receptor MET, en pacientes con tumores malignos sólidos en fase avanzada
DU176b-D-U311
Estudio fase IIIB, prospectivo, randomizado, abierto que evalúa la eficacia y seguridad de Heparina/Edoxaban versus Dalteparina en tromboembolismo venoso asociado con cáncer.
Birinapant, Azacitidina. Síndrome Mielodisplásico/LMC
Estudio clínico de fase II multicéntrico, randomizado, doble ciego, controlado con placebo de Azacitidina con o sin Birinapant en pacientes con Síndrome mielodisplásico de alto riesgo o Leucemia Mielomonocítica crónica
Deferasirox. Sindrome mielodisplásico. CICL670F2201
Estudio fase II, multicéntrico, aleatorizado, abierto, con dos brazos de tratamiento para investigar los beneficios de una formulación mejorada de deferasirox (comprimido recubierto)
Ensayo en fase III, doble ciego, multicéntrico que compara magrolimab en combinación con azacitidina frente a azacitidina más placebo en pacientes que no han sido tratados con alto riesgo de síndrome mieloidedisplásico
ENHANCE
Ensayo fase I, abierto, de búsqueda de dosis de CC-90009, un nuevo fármaco modulador de cereblon E3 ligasa, en sujetos con leucemia mieloide aguda en recaída o refractaria o con síndrome mielodisplásicos de alto grado riesgo en recaída o refractarios
CC-90009-AML-001

Most cases of myelodysplastic syndrome have no known cause, but some factors have been determined to increase the risk. Advancing age is perhaps the most common risk factor, since it rarely occurs in people under the age of 60. Other risk factors include smoking, long-term exposure to benzene and prior treatment with chemotherapy or radiation.

Over the last few years, the care of patients with myelodysplastic syndrome and the understanding of this disease have improved significantly. This has resulted in the development of new therapies.

 

Lower risk myelodysplastic syndrome patients are treated initially for the specific complications of the disease, such as anemia and low blood counts. If there are indications for more aggressive therapy, strategies that are now considered standard of care include the hypomethylating agents (5-azacitidine and decitabine) and lenalidomide.

 

Higher risk myelodysplastic syndrome patients usually need more aggressive therapy but much depends on the age and condition of the patient. Younger patients with high risk disease are considered for front-line chemotherapy approaches followed perhaps by allogeneic stem cell transplantation. For older patients, who constitute the majority, intensive chemotherapy is rarely considered. Instead, our strategy focuses on development of active and safe treatments for newly diagnosed patients as well as those who have failed the standard of care based therapies.

 

Myelodysplastic syndrome is one of the main areas of research of the Department of Leukemia. This is demonstrated by the large number of clinical trials specific for patients with myelodysplastic syndrome and by the extensive basic and translational research exemplified by our NIH funded MDS P01 grant, now in its fourth year, to increase our understanding of the disease. Approximately 300 patients with myelodysplastic syndromeare evaluated in our center per year by a group of physicians completely dedicated to the care of these patients. This care is provided in close collaboration with the referring physicians, a key aspect for the long-term follow up of these patients who will require extensive care during the course of their myelodysplastic syndrome.