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What is Myelodysplastic Syndrome?

The myelodysplastic syndromes (MDS) comprise a heterogeneous group of cancerous stem cell disorders characterized by dysplastic (abnormal) and ineffective blood cell production and a variable risk of transformation to acute leukemia. These disorders may occur de novo or arise years after exposure to potentially mutagenic therapy (eg, radiation exposure, chemotherapy).

The risk of developing MDS increases with age.  Signs and symptoms at presentation of MDS are non-specific. Many patients are asymptomatic, with the diagnosis established upon routine laboratory screening. Others present with symptoms resulting from a previously unrecognized anemia, including fatigue, weakness, exercise intolerance, angina, dizziness, cognitive impairment, or an altered sense of well being.

While low white blood cells count is largely responsible for the high incidence of infection in MDS, granulocyte dysfunction (eg, impaired microbial killing) may also contribute. Bacterial infections predominate; the skin is the most common site of infection.

The 5q- syndrome — There is a special subtype of MDS called 5q minues. It can be present in patients otherwise diagnosed as having refractory anemia; however, its karyotypic and clinical distinctiveness sets it apart from the other

In a study of 88 consecutive patients defined by the 2008 WHO criteria, a relatively unique pattern was seen:

  • Female predominance with a median age at diagnosis of 74 years
  • Transfusion-dependent anemia
  • Low incidence of neutropenia, thrombocytopenia, infection, and bleeding
  • Normal or increased platelet counts along with bone marrow hyperplasia of hypolobulated micromegakaryocytes
  • Low incidence of transformation into acute leukemia

How to diagnose MDS: Other important causes of low blood counts need to be ruled out including poor nutritional status, alcohol and drug use, copper level, medications such as valproic acid, occupational exposure to toxic chemicals, prior treatment with antineoplastic agents or radiotherapy, and risk factors for and/or treatment of human immunodeficiency virus (HIV) infection should be elicited and vitamin B12 and folate deficiency must be excluded.

Your doctor will examine your blood under the microscope and ultimately will require a bone marrow biopsy.

 

Prognostication is based upon the IPSS score:

Percentage of Bone Marrow Blasts

< 5 percent (0 points)

5 to 10 percent (0.5 points)

11 to 20 percent (1.5 points)

21 to 30 percent (2.0 points)

Karyotype

Normal, Y-, 5q-, 20q- (0 points)

Abnormal chromosome 7 or 3 or more abnormalities (1.0 points)

All other cytogenic abnormalities (0.5 points)

Cytopenias (defined as hemoglobin < 10 g/dL, absolute neutrophil count < 1800/microL, platelet count < 100,000/microL)

No cytopenia or cytopenia of 1 cell type (0 points)

Cytopenia of 2 or 3 cell types (0.5 points)

You are given a score based up on the blasts, chromosome analysis and number of reduced blood cells and your doctor will be able to roughly prognosticate the aggressiveness of the MDS.

 

Treatment of MDS:

Treatment options for patients with MDS typically fall into one of three categories:

  • Supportive care includes the use of antibiotics for infection and red cell and platelet transfusions in the setting of symptomatic anemia and thrombocytopenia, respectively. Prophylactic antibiotics are generally not helpful and this strategy may select for antibiotic resistance. Supportive care is an important adjunct to the management of all patients with MDS and can be considered as the sole treatment modality for a subset of patients with lower risk MDS.
  • Low intensity therapies include hematopoietic growth factors, DNA hypomethylating agents (vidaza or decitabine), immunosuppressive therapy, and lenalidomide. These can be administered in the outpatient setting and have a low risk of treatment-related morbidity and mortality. Low intensity treatments can improve symptoms and quality of life, but are not curative.
  • High intensity therapies include intensive combination chemotherapy and allogeneic hematopoietic cell transplantation (HCT). They require hospitalization and entail significant risk of treatment-related mortality. However, these treatments may improve blood counts more quickly than less intensive therapy, reduce the risk of death from MDS, and may alter the MDS disease course.

 Thanks uptodate.com

Here, Dr. Tony Talebi discusses “What is Myelodysplastic Syndrome?” with Dr. Byrnes, professor of Hematology at the University of Miami.   Discussion includes MDS prognosis, what causes MDS, signs of MDS, MDS symptoms, MDS, MDS stage 4, treatment of MDS, MDS association, stage four MDS, small cell MDS, symtoms of MDS, causes of MDS, MDS chemotherapy, what is MDS, MDSs, MDS information, MDS prevention, stage 4 MDS, information about MDS, stage iv MDS, MDS signs, MDS symptom, is MDS curable, survival rates for MDS, symptoms of MDS, MDS survivors, MDS symptons, MDS survival, treatments of MDS, symptons of MDS, MDS statistics,  MDS, chemo for MDS, MDS survival rate, large cell MDS, effects of MDS, MDS screening, MDS diagnosis, MDS society, MDS clinical trials, MDS metastasis, survival rate MDS, symptom of MDS, info on MDS, new treatments for MDS, how common is MDS, vidaza, blood cell growth factors, stem cell transplant for MDS, blood transfusions for MDS,  IPSS score and treatment of MDS.


Dr Byrnes' crendentials:

Professor Hematology and Chief of Hematology/Oncology at the Miami VA Hospital.


Education

  • College of the Holy Cross
  • Undergraduate
  • Tufts University School of Medicine
    United States
  • Graduate
  • State University of NY at Buffalo
    United States
  • Internship
  • State University of NY at Buffalo
    United States
  • Residency
  • University of Miami
    United States
  • Fellowship