title: Hypogammaglobulinemia and reduced numbers of B-cells in children with myelodysplastic syndrome.
abstract: BACKGROUND
  Immunodeficiency in pediatric patients with myelodysplastic syndrome (MDS) has not been described. We report the clinical course of three children with MDS, hypogammaglobulinemia, and reduced numbers of B-cells and B-cell precursors.
  
  
  METHODS
  Three patients with recurrent infection who were younger than 1-year-old had MDS of the refractory anemia (RA) subtype diagnosed. All had reduced numbers of circulating B-cells and hypogammaglobulinemia. In two patients, cytogenetic studies revealed a monosomy 7 karyotype and bone marrow studies showed decreased numbers of CD34+ progenitor cells and CD 19+ B-cells. Both patients had prolonged courses (7 yrs 10 mos and 6 yrs 9 mos) characterized by recurrent infection and slowly progressive pancytopenia. Both received allogeneic bone marrow transplantation (BMT). The third patient had normal cytogenetic studies and a normal number of CD34+ progenitors but decreased CD19+ B-cells in the bone marrow. She had a stable course with refractory anemia over the course of 7 years.
  
  
  CONCLUSIONS
  Pediatric patients with MDS may have hypogammaglobulinemia and reduced numbers of B-cells. These findings do not preclude a relatively stable and prolonged clinical course. Children with newly diagnosed MDS should have an immunologic evaluation in addition to their hematologic assessment.
authors.affiliation: Department of Hematology/Oncology, St Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
authors.forename: K
authors.initials: K
authors.lastname: Srivannaboon
authors.forename: M E
authors.initials: ME
authors.lastname: Conley
authors.forename: E
authors.initials: E
authors.lastname: Coustan-Smith
authors.forename: W C
authors.initials: WC
authors.lastname: Wang
mesh_terms: D000361:Agammaglobulinemia / Q000209:etiology*; D000753:Anemia, Refractory / Q000097:blood / Q000150:complications* / Q000235:genetics / Q000276:immunology / Q000628:therapy; D001402:B-Lymphocytes; D001803:Blood Transfusion; D016026:Bone Marrow Transplantation; D016516:CD4-CD8 Ratio; D019070:Cell Lineage; D002648:Child; D002897:Chromosomes, Human, Pair 7; D005260:Female; D006412:Hematopoietic Stem Cells; D006801:Humans; D016867:Immunocompromised Host; D016130:Immunophenotyping; D007239:Infections / Q000209:etiology; D018655:Lymphocyte Count; D008231:Lymphopenia / Q000209:etiology*; D008297:Male; D009006:Monosomy; D012008:Recurrence