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Neck Swelling and Fever

  • Sowmya ShashidharaEmail author
  • H. Koushik
  • Sangita Trivedi
Chapter

Abstract

  • Clinical suspicion of bone marrow failure syndrome should be raised in any child presenting with fever, weight loss, organomegaly with neutropenia and peripheral blood pancytopenia

  • A cellular bone marrow with dysplastic cells and peripheral blood dysgranulopoiesis in the above clinical setting is suggestive of myelodysplastic syndrome (MDS)

  • GATA2 deficiency results from a genetic aberration found in congenital types of myelodysplastic syndrome

  • Acquired myelodysplastic syndrome is associated with previous chemotherapy with cyclophosphamide and etoposide which leads to activation of proto-oncogenes

  • Hematopoietic stem cell transplantation is the definitive treatment for MDS

  • Bone marrow blast count and thrombocytopenia are the factors associated with outcome in childhood MDS

Keywords

GATA2 deficiency Myelodysplastic syndrome Hematopoietic stem cell transplantation 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Sowmya Shashidhara
    • 1
    • 2
    Email author
  • H. Koushik
    • 1
  • Sangita Trivedi
    • 3
  1. 1.Department of PediatricsKasturba Medical College, Manipal UniversityManipalIndia
  2. 2.Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA)Universal Scientific Education and Research Network (USERN)ManipalIndia
  3. 3.Department of PediatricsGovernment Medical College, Surat, Veer Narmad South Gujarat UniversitySuratIndia

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