Myelodysplastic Syndrome (MDS), Therapies for Myelodysplastic Syndromes, Risk Category and Current Treatment Strategies

Myelodysplastic Syndrome (MDS), Therapies for Myelodysplastic Syndromes, Components of NCCN Guidelines

Therapies for Myelodysplastic Syndromes, NCCN Guidelines

Myelodysplastic Syndrome (MDS), Therapies for Myelodysplastic Syndromes, Therapeutic Approaches for MDS Treatment

Myelodysplastic Syndrome (MDS), Therapies for Myelodysplastic Syndromes, Classification and Prognosis

Myelodysplastic Syndrome (MDS), Therapies for Myelodysplastic Syndromes, Classification and Prognosis: French-American-British (FAB)

Myelodysplastic Syndromes: World Health Organization (WHO) Classification

Myelodysplastic Syndrome (MDS), Therapies for Myelodysplastic Syndromes, International Prognostic Scoring System (IPSS)

International Prognostic Scoring System (IPSS)

Supportive Care

• Current Treatment Strategies
  • Low risk patients (Low/Intermediate-1)
    • Hematological improvement
    • Low intensity therapy
  • High risk patients (Intermediate-2/High)
    • Change disease natural history
    • High intensity therapy

• Three Major Elements
  • Algorithm or decision pathway outlining care management
  • Manuscript on important issues related to the algorithm
  • References providing data for the basis of the recommendations
• Balance sum of benefits vs. risks
• Systematically developed through an evidence-based approach

• Comprehensive diagnostic, treatment and supportive care guidelines
• Systematically developed statements
• Enhance clinical decision-making

Decision Factors for MDS Treatment:

• MDS classification and prognosis
• Cytogenic abnormalities, i.e. 5q deletion
• Patient age
• Patient health

Classification and Prognosis (MDS)

• MDS: Heterogeneous group of acquired bone marrow disorders
• Classification and therapeutic decision making is complex
• Systems of Classification and Prognosis:
  • French-American-British (FAB) Classification System
  • World Health Organization (WHO) Classification System
  • International Prognostic Scoring System (IPSS)

Classification and Prognosis (MDS)

• French-American-British (FAB) Classification System
  • Percentage of blast cells in the peripheral blood and bone marrow
  • Cytopenias and dysplasia
  • Monocytosis
  • Ringed Sideroblasts

Classification and Prognosis

• French-American-British (FAB) Classification System
  • RA and RARS
    • Low risk patients
    • Relatively good prognosis
    • 4-5 year median survival
    • Acute myelogenous leukemia rate of < 25%

Classification and Prognosis

• French-American-British (FAB) Classification System
  • RAEB
    • Intermediate group
    • Median survival of 1 year
  • RAEB-t
    • High rate of transformation
    • Poor prognosis
    • Median survival of a few months

World Health Organization (WHO) Classification

• Update to the FAB classification system
• Differences between the FAB and WHO classification systems
  • Acute leukemia ≥ 20% vs. 30% blast cells
  • Refinement of refractory cytopenia and refractory anemia categories
  • Isolated 5q deletion

Lower risk patients

• Low or intermediate-1 risk categories

Higher risk patients

• Intermediate-2 or high risk categories

Within the IPSS system, survival is additionally affected by patient age.

Patients are separated into two categories:
≤ 60 years of age
> 60 years of age.

International Prognostic Scoring System (IPSS)

• Diverse genotypes and impact on outcomes
  • Including Y, 5q and 20q deletions
• Blast percentages
• Number of lineages with cytopenias

International Prognostic Scoring System (IPSS)

• Including Y, 5q and 20q deletions
• Blast percentages
• Number of lineages with cytopenias

G. Garcia-Manero and H.M. Kantarjian.  New Investigational Strategies in Patients with Myelodysplastic Syndromes.  In The Myelodysplastic Syndromes. Pathobiology and clinical Management.  Edited by J.M. Bennett.  Marcel Dekker, Inc. New York, N.Y. 2002.  Chapter 15, Page 399.

D.P. Steensma and J.M. Bennett.  The Myelodysplastic Syndromes: Diagnosis and Treatment.  Mayo Clin Proc.  81(1):104-130, 2006.

D.P. Steensma and J.M. Bennett.  The Myelodyplastic Syndromes: Diagnosis and Treatment. Mayo Clin Proc. 81(1):104-130, 2006. Page 112 (or 9).

D.P. Steensma and J.M. Bennett.  The Myelodysplastic Syndromes: Diagnosis and Treatment. Mayo Clin Proc. 81(1):104-130, 2006. Page 112 (or 8).

D.P. Steensma and J.M. Bennett.  The Myelodysplastic Syndromes: Diagnosis and Treatment. Mayo Clin Proc. 81(1):104-130, 2006. Page 112 (or 8).

D.P. Steensma and J.M. Bennett.  The Myelodysplastic Syndromes: Diagnosis and Treatment. Mayo Clin Proc. 81(1):104-130, 2006. Page 112 (or 8).

National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology.  Myelodysplastic Syndromes. V.1.2009.  Page MS-5.

National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology.  Myelodysplastic Syndromes. V.1.2008.  Page MS-6.

National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology.  Myelodysplastic Syndromes. V.1.2009.  Page MS-8.

NCCN = National Comprehensive Cancer Network

FAB = French-American-British Classification System

WHO = World Health Organization

CMML-1 = Chronic Myelomonocytic Leukemia-1

RCMD = Refractory Cytopenia with Multilineage Dysplasia

CML = Atypical Chronic Myeloid Leukemia

JMML = Juvenile Myelomonocytic Leukemia

IPSS = International Prognostic Scoring System