Hypogammaglobulinemia and reduced numbers of B-cells in children with myelodysplastic syndrome.
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.
Srivannaboon
K
K
Department of Hematology/Oncology, St Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
Conley
M E
ME
Coustan-Smith
E
E
Wang
W C
WC
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