eMedicine Specialties > Pediatrics: General Medicine > Hematology

Myelodysplasia: Follow-up

Author: Sharon M Castellino, MD, FAAP, Assistant Professor, Department of Pediatrics, Division of Pediatric Hematology-Oncology, Wake Forest University Health Sciences
Coauthor(s): Timothy P Cripe, MD, PhD, Associate Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center; Scott C Howard, MD, Associate Professor, University of Tennessee College of Medicine; Associate Member, Department of Oncology, Director of Clinical Trials, International Outreach Program, St Jude Children's Research Hospital
Contributor Information and Disclosures

Updated: Apr 11, 2008

Follow-up

Further Inpatient Care

  • Inpatient admission for patients with myelodysplasia syndrome (MDS) is usually for treatment of fever during periods of neutropenia. These episodes require aggressive evaluation for a source of infection and empiric coverage with broad-spectrum antibiotics against gram-negative rods. Blood and urine should be cultured for bacteria and for fungus, depending on the duration of symptoms.
  • Inpatient care at a designated center is also needed for stem cell transplantation.

Further Outpatient Care

  • Outpatient follow-up care depends on the degree of anemia and thrombocytopenia. Close follow-up is warranted, as progression to frank AML can occur over weeks to months.
  • Transfusion support is now manageable in the outpatient setting. Packed RBCs and platelets need to be leukofiltered and irradiated. Donor exposure to platelets should be minimized with pheresis and single-donor products whenever possible. This minimizes the risk of development of alloimmunization and the risk of the patient becoming refractory to transfusions.

Inpatient & Outpatient Medications

  • Patients are often placed on Pneumocystis carinii pneumonia (PCP) prophylaxis because of their degree of immunosuppression. Trimethoprim-sulfamethoxazole (Bactrim, Septra) is commonly used on a 3-times-per-week schedule. In patients allergic to Bactrim or in cases of Bactrim-related myelosuppression, oral atovaquone or aerosolized pentamidine is effective on a monthly schedule.

Transfer

  • Patients should be referred to centers with established stem cell transplant programs and experience in treating myelodysplasia syndrome and other hematologic malignancies.

Complications

  • Infection: Patients with myelodysplasia syndrome may have increased risk for infection due to depressed granulocyte number and function. Even in cases of normal neutrophil number, neutrophils may exhibit decreased myeloperoxidase and microbicidal activity. Granulocytes may exhibit poor adhesion, chemotaxis, phagocytosis, and decreased microbicidal activity. Patients are extremely susceptible to life-threatening gram-negative rod and fungal infections.
  • Bleeding: Patients often have thrombocytopenia and resultant hemorrhage. Platelet dysfunction may occur in myelodysplasia syndrome. Patients require frequent transfusions as the bone marrow becomes increasingly hypoplastic.
  • Anemia: In rare circumstances, iron overload is a complication of chronic RBC transfusion and may necessitate iron chelation therapy.

Prognosis

  • Findings associated with a poorer prognosis in childhood myelodysplasia syndrome include RAEB and RAEBT. Age younger than 2 years and hemoglobin F levels greater than 10% have proven in several series to be unfavorable features in patients. This encompasses most children with JMML. This group of patients has been largely refractory to all therapy. Patients with major chromosomal abnormalities, such as monosomy 7, have a dismal course unless they proceed to allogeneic stem cell transplantation.
  • Association with Down syndrome and trisomy 8 has conferred favorable prognosis in United Kingdom experience.
  • Without transplantation, the median survival time in children with the most common subtypes for this age group (RAEB, RAEBT) is less than 1 year.

Patient Education

  • Patient education should relate to prevention and treatment of complications of thrombocytopenia and neutropenia, as outlined in Treatment. In cases in which patients have a central venous access device, parents must be educated with regard to its care.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Carmen Arkansas, MD, and Glenda Grawe, MD, to the development and writing of this article.



More on Myelodysplasia

Overview: Myelodysplasia
Differential Diagnoses & Workup: Myelodysplasia
Treatment & Medication: Myelodysplasia
Follow-up: Myelodysplasia
References
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Further Reading

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Keywords

myelodysplasia, myelodysplasia syndromes, MDS, myelodysplastic syndromes, MDSs, preleukemia syndromes, dysmyelopoietic syndromes, hematopoietic dysplasia, refractory dysmyelopoietic anemia, monosomy 7 syndrome, refractory anemia, juvenile chronic myelogenous leukemia, JCML, hematopoiesis, refractory anemia with excess of myeloblasts, subacute myeloid leukemia, oligoleukemia, odoleukemia, stem cell disorder
 
cytopenia, acute nonlymphocytic leukemia, ANLL, neurofibromatosis, NF, neutropenia, thrombocytopenia, juvenile myelomonocytic leukemia, JMML, Fanconi anemia, severe congenital neutropenia, Kostmann syndrome, Down syndrome, Noonan syndrome, Shwachman-Diamond disease, Diamond-Blackfan anemia, Dubowitz syndrome, Bloom syndrome, Poland syndrome, ataxia telangiectasia, bone marrow failure, dyskeratosis congenita, bone marrow transplantation, graft versus host disease, graft rejection, juvenile chronic myelogenous leukemia, JCML, splenomegaly

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Contributor Information and Disclosures

Author

Sharon M Castellino, MD, FAAP, Assistant Professor, Department of Pediatrics, Division of Pediatric Hematology-Oncology, Wake Forest University Health Sciences
Sharon M Castellino, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Coauthor(s)

Timothy P Cripe, MD, PhD, Associate Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center
Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Scott C Howard, MD, Associate Professor, University of Tennessee College of Medicine; Associate Member, Department of Oncology, Director of Clinical Trials, International Outreach Program, St Jude Children's Research Hospital
Scott C Howard, MD is a member of the following medical societies: American Society of Clinical Oncology and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

Medical Editor

Sharada A Sarnaik, MB, BS, Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Attending Hematologist/Oncologist, Children's Hospital of Michigan
Sharada A Sarnaik, MB, BS is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research
Disclosure: Nothing to disclose.

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Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

James L Harper, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center
James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society
Disclosure: Nothing to disclose.

CME Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida, Clinical Professor, Department of Pediatrics, UNC, Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD, King Fahd Professor of Pediatric Oncology, Department of Oncology, Division of Pediatric Oncology, Johns Hopkins University School of Medicine
Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Clinical Oncology, American Society of Hematology, and American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.

 
 
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