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Myelodyplastic Syndromes Paul J. Shami, M.D. Professor of Hematology, University of Utah Member, Huntsman Cancer Institute

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Page 1: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

Myelodyplastic Syndromes

Paul J. Shami, M.D.

Professor of Hematology, University of Utah Member, Huntsman Cancer Institute

Page 2: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

Objectives

• Define Myelodysplastic Syndromes (MDS) • Explain how MDS are diagnosed and

classified • Discuss the different treatment options • Identify patient education and support

resources • Better prepare patients to discuss their

diagnosis, treatment, and care with their physicians, team, family, and friends

Page 3: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

Terminology

•  Cancer •  Benign •  Malignant •  Metastatic

•  Blood (bone marrow)-related cancers •  Leukemia •  Lymphoma •  Myeloma •  Myelodysplastic syndromes •  Myeloproliferative disorders

•  Types of leukemia •  Acute vs. Chronic •  Lymphoid vs. Myeloid

Page 4: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS: Bone Marrow-Related Cancers

Myeloid Cells Lymphoid Cells

Page 5: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

Myelodysplastic Syndromes

• Clinical diseases characterized by low blood counts (anemia, low WBC, low platelets)

• Bone marrow usually shows increased number of cells • Can develop into AML

Page 6: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS Epidemiology

• ~ 20,000 estimated new cases/year in US • Predominantly a disease of the elderly

•  Median age > 60 •  Incidence greater in men than women •  Incidence increases with age

• Median survival varies depending on risk category

Page 7: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - Symptoms

•  Many patients have no apparent symptoms, but are diagnosed after routine laboratory tests uncover abnormalities in the circulating blood cells

•  Fatigue is the most common symptom of MDS •  Early symptoms of MDS may include:

•  Bruising •  Bleeding •  Shortness of breath •  Rapid heart rate •  Weight loss •  Fever •  Loss of appetite

Page 8: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - Risk factors

• Cause of MDS unknown • Damage to the DNA of bone marrow cells • Environmental

• Certain chemicals (Benzene) • Radiation exposure • Chemotherapy

Page 9: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - Diagnosis • History/Physical Exam • Blood tests

•  Blood count •  Chemistries •  Iron studies •  B12/Folate •  Erythropoietin level

• Bone marrow biopsy •  Morphology (examine slides under microscope) •  Flow cytometry (check for abnormal cells) •  Cytogenetics/FISH (chromosome test) •  Molecular studies (DNA mutations)

Page 10: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - Complications

• Bleeding •  Low platelet count

• Infections •  Low levels of normal white blood cells that

fight infections

• Acute Myeloid Leukemia

Page 11: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS Classification

• French American British (FAB) •  no longer used

• World Health Organization (WHO) •  currently used and regularly updated

• International Prognostic Scoring System – Revised (IPSS-R) •  used for prognostication and treatment planning

Page 12: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - WHO classification

Page 13: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS – IPSS-R

• Patients are stratified into five

risk groups according to survival and risk of AML transformation

• Scoring system based on % of bone marrow blasts, chromosomes and severity of blood count abnormalities

Page 14: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS – IPSS-R

Blood 120: 2454-2465, 2012

Page 15: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

IPSS-­‐R  Survival  (n=7012)  

Months

Page 16: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

IPSS-­‐R  Freedom  from  AML  Transforma@on  

Months

Page 17: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - Management

1- Determine disease risk based on IPSS-R score. 2- Consider observation to determine pace of disease progression. 3- Stratify patients according to risk. 4- Individualize approach based on patient’s age, performance status, health, etc…

Page 18: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - Management Low risk disease

1- Treat if clinically significant low blood counts. 2- Transfusion support as needed. 3- Iron chelation therapy if indicated. 4- If 5q- present - treat with Lenalidomide (Revlimid). 5- If 5q- absent - consider treatment with growth factors (erythropoietin +/- G-CSF). 6- If no response to growth factors, consider hypomethylating agents (decitabine, azacitidine). 7- Determine if patient is eligible for immunosuppressive therapy (cyclosporine, ATG) and treat accordingly.

Page 19: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

MDS - Management High risk disease

1- Azacitidine or decitabine. 2- Transplant if patient is candidate.

Page 20: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

Talking With Your Team: What Position Do You Play?

•  Ask questions about your disease and treatment •  Keep your doctors’ appointments •  Keep your doctor & nurse informed of side effects •  Inform your doctor & nurse before taking other

medications •  Avoid supplements •  Avoid alcohol •  Look at your attitude and explore support options

Page 21: Professor of Hematology, University of Utah …...Professor of Hematology, University of Utah Member, Huntsman Cancer Institute Objectives • Define Myelodysplastic Syndromes (MDS)

Patient Education and Support Services

•  Myelodysplastic Syndromes Foundation •  www.mds-foundation.org

•  The Leukemia & Lymphoma Society •  www.lls.org

•  National Cancer Institute •  www.cancer.gov


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Ruben A. Mesa, MD Professor of Medicine Chair, Division of Hematology & Medical Oncology

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Clinical Trials Jame Abraham, M.D Associate Professor of Medicine Chief, Section of Hematology- Oncology

Todd Campbell Associate Professor Science Education Utah State University

Bone Marrow Failure - Cancer Medicine & Hematology · Bone Marrow Failure Robert A. Brodsky, MD Johns Hopkins Family Professor of Medicine and Oncology. Director, Division of Hematology

Advances in hematology and oncology - acponline.org fileAdvances in hematology and oncology for 2018 G Weldon Gilcrease III, MD University of Utah. ... Blood Journal 2013. JCO. July

HEMATOLOGY AND PEDIATRIC ONCO-HEMATOLOGY DEPARTMENT · HEMATOLOGY AND PEDIATRIC ONCO-HEMATOLOGY DEPARTMENT ... Hematology and Pediatric onco-Hematology department HEAD ... elena Maggioni

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Normocytic Normochromic Anemias M H Qari, MD, FRCPA Associate Professor of Hematology

Hematology for Surgeons Michael H. Rosove, MD Clinical Professor of Medicine UCLA Division of Hematology-Oncology November 13, 2013

Hematology Emergencies: Problems with Platelets Emergencies: Problems with Platelets Christian Cable, MD, FACP Associate Professor of Medicine Division of Hematology & Oncology Texas

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REGISTRATION Hematology/Oncology Update for the Primary€¦ · 4/10/2013  · Rajesh Sehgal, MD, FACP Assistant Professor, Section of Hematology/Oncology Marshall University JCESOM

Ahmad O. Hammoud MD, MPH Assistant Professor Obstetrics and Gynecology Reproductive Endocrinology and Infertility University of Utah Medical Director Utah

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Lymphoma Farjah Hassan AlGahtani Assistant Professor, Consultant Hematology Director of Transfusion Medicine and Blood Bank

Kelly Bricker GSTC Associate Professor, University of Utah [email protected]

By Magy Abdelwahab Professor of Pediatric Hematology Cairo University Pediatric Hospital

Hematology Alterations: Altered Leukocyte …courses.justice.eku.edu/NSC834a/ppt/Lesson4/834 Hematology 3 WBC...Mary DeLetter, PhD, RN. Associate Professor. Dept. of Baccalaureate

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Anemia: Overview and Select Cases Molly W. Mandernach, MD, MPH Assistant Professor Division of Hematology/Oncology

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Adler - PP BFS... · William Adler MD Hematology/Oncology Medical and Clinical Research Director MMC Cancer Center Assistant Professor, Hematology & Oncology Department of Internal

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HEMATOLOGY CLINICAL PEARLS - Internal Medicine · PDF fileHEMATOLOGY CLINICAL PEARLS Rakhi Naik, MD, MHS Assistant Professor of Medicine & Oncology, Division of Hematology

Introduction to thrombosis [email protected] Assistant Professor of Medicine Hematology/Oncology2014

Ganesh Gopalakrishnan Associate Professor Computer Science University of Utah

DIVISION OF HEMATOLOGY-ONCOLOGY - UPMC · PDF file˜˚˛˜˝˙ˆ˝ˇ˘ ˜ ˚ ˘ ˙ UPMC DIVISION OF HEMATOLOGY-ONCOLOGY Annie Im, MD Assistant Professor of Medicine Associate Program

COLORECTAL CANCER Charles Lopez, M.D., Ph.D. Associate Professor of Medicine Hematology and Medical Oncology May 2009

Thrombotic Complications in Hematologic Malignancies Hau C. Kwaan, MD, FRCP Marjorie C Barnett Professor of Hematology-Oncology Division of Hematology

Adult Hematology and Oncology...Adult Hematology and Oncology Dr. Ahmed Almohanad Nadim Absi Assistant Professor Hematology/Oncology Consutant Hematology Oncology BN: 9196197 [email protected]