[Neutropenia in patient with X-linked hyper-IgM syndrome].
The X-linked form of hyper-IgM syndrome (HIGM1) is a rare disorder characterized by the inability of B cells to undergo isotype switch by a deficiency of CD40 ligand (CD40L) on activated T lymphocytes. The patients suffer from recurrent infections not only due to a lack of B lymphocyte activation but also due to defect of T lymphocyte functions. In addition, neutropenia is frequently accompanied by these symptoms. A patient with HIGM1, we experienced, suffered from recurrent infections and neutropenia. But he had a normal number of hematopoietic stem cell by the in vitro colony forming assay. CD34+ myeloid stem cell has been known to express CD40. We speculated by these facts that myeloid cell numbers are regulated by CD40-CD40L interaction.
Department of Pediatrics, Kumamoto University Medical School.
M
M
Iwata
H
H
Nunoi
S
S
Nonoyama
M
M
Shimadzu
S
S
Higuchi
Y
Y
Yanabe
M
M
Migita
N
N
Adachi
I
I
Matsuda
D000293:Adolescent; D001402:B-Lymphocytes / Q000276:immunology; D001483:Base Sequence; D019013:CD40 Antigens / Q000235:genetics; D008040:Genetic Linkage; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006412:Hematopoietic Stem Cells; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000628:therapy; D007075:Immunoglobulin M; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000628:therapy; D008024:Ligands; D008297:Male; D008969:Molecular Sequence Data; D009503:Neutropenia / Q000209:etiology* / Q000628:therapy; D011994:Recombinant Proteins / Q000627:therapeutic use; D013601:T-Lymphocytes / Q000276:immunology
D019013:CD40 Antigens; D007075:Immunoglobulin M; D008024:Ligands; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor