eMedicine Specialties > Hematology > Stem Cells and Disorders

Myelodysplastic Syndrome: Follow-up

Author: Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Coauthor(s): Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Contributor Information and Disclosures

Updated: Apr 28, 2009

Follow-up

Further Inpatient Care

  • Bone marrow transplantation with a matched allogeneic or syngeneic donor is used in patients with poor prognoses or late-stage myelodysplastic syndrome (MDS) who are aged 55 years or younger and have an available donor. Among selected patients with less advanced/low-risk myelodysplastic syndrome (MDS) (<5% marrow myeloblasts), a 3-year survival of 65-75% is achievable with HLA-matched related and unrelated donors. Because hematopoietic stem cell transplantation (HSCT) is potentially a curable therapy option, the timing of the procedure may be important in this subgroup of patients.
  • Compared with patients with de novo acute myeloid leukemia transplanted in first remission, patients with myelodysplastic syndrome (MDS) experience higher mortality rates associated with the procedure (21-30% vs 10%), lower disease-free survival rates, and higher relapse rates (70% vs 40%). Among the patients with more advanced/high-risk disease (>5% marrow myeloblasts and high IPSS scores), the probability of posttransplant relapse ranges from 10-40%; as a result, relapse-free survival is inferior in this group, which needs this therapy because of the bad prognosis.
  • Because most patients are elderly and only a few young patients myelodysplastic syndrome (MDS) will have a matched donor, the use of bone marrow transplantation is limited.
  • Recently, the use of nonmyeloablative (mini) bone marrow transplantation and reduced-intensity conditioning regimens has been used in elderly patients as old as 75 years with some success. This approach is still considered experimental and should be performed in a clinical trial setting.

Transfer

  • Because most treatment for myelodysplastic syndrome (MDS) is not standard and is considered experimental, referral to a tertiary care center is often necessary.

Complications

  • The disease itself is associated with complications associated with severe cytopenias. Other complications of myelodysplastic syndrome (MDS) are as follows:
    • The development of myelofibrosis can accelerate a decline in blood counts and an increase in transfusion requirements.
    • Transformation to acute leukemia accelerates the development of complications such as anemia, bleeding, and infections.
    • Patients with an enlarged spleen may have complications related to spontaneous rupture and intra-abdominal exsanguination.

Prognosis

  • To improve prognostic classification, the MDS Risk Analysis Workshop has developed the IPSS. It takes into account the cytopenias, percentage of bone marrow blasts, and cytogenetics.
    • Those with a good prognoses include patients with single or mild cytopenias, normal chromosomes or a single chromosomal abnormality (except those involving chromosome 7), and greater than 10% myeloblasts in the bone marrow. These patients have mean survival of 18-24 months or longer.
    • Patients with pancytopenia requiring RBC or platelet transfusions, patients with chromosome 7 or multiple abnormalities, and patients with greater than 10% myeloblasts in the bone marrow have a 6- to 12-month survival rate.

Miscellaneous

Medicolegal Pitfalls

  • If underlying dysplastic changes were missed initially, thrombocytopenia as the presenting symptom may be mistaken for immune thrombocytopenia.
  • Splenectomy for the cytopenia in a patient with myelodysplastic syndrome (MDS) is dangerous and fraught with complications and, thus, is not recommended.
 


More on Myelodysplastic Syndrome

Overview: Myelodysplastic Syndrome
Differential Diagnoses & Workup: Myelodysplastic Syndrome
Treatment & Medication: Myelodysplastic Syndrome
Follow-up: Myelodysplastic Syndrome
Multimedia: Myelodysplastic Syndrome
References
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References

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Further Reading

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Keywords

myelodysplastic syndrome, dysmyelopoietic syndrome, acute myeloid leukemia, MDS, preleukemia, refractory dysmyelopoietic anemia, smoldering leukemia, subacute myelogenous leukemia, dysmyelopoiesis, refractory anemia, RA, RA with ringed sideroblasts, RARS, RA with excess blasts, RAEB, RAEB in transformation, RAEB-T, anemia, blood disease,

hematopoietic disorders, leukemia, acute leukemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, chronic myelomonocytic leukemia, CMML, hematopoietic stem cell injury, apoptosis, programmed cell death, petechiae, thrombocytopenia, neutropenia,

epistaxis, gum bleeding, hemoptysis, hematuria, enlarged spleen, pneumonias, urinary tract infections, secondary acute leukemia, primary MDS, secondary MDS, leukemogenic chemicals, insecticides, weed killers, fungicides

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Contributor Information and Disclosures

Author

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Coauthor(s)

Ulrich Woermann, MD, Consulting Staff, Division of Instructional Media, Institute for Medical Education, University of Bern, Switzerland
Disclosure: Nothing to disclose.

Medical Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University
Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Troy H Guthrie, Jr, MD, Director of Cancer Institute, Baptist Medical Center
Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University
Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians
Disclosure: Nothing to disclose.

 
 
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