Immune status of Fanconi anemia patients: decrease in T CD8 and CD56dim CD16+ NK lymphocytes.
Fanconi anemia (FA), a rare genetic disease in which patients' life is compromised mainly by hematological abnormalities and cancer prone, seems to be affected by subtle immune cell irregularities. Knowing that FA presents developmental abnormalities and, based on recent reports, suggesting that natural killer (NK) CD56(dim) and NK CD56(bright) correspond to sequential differentiation pathways, we investigated if there were changes on the total number of NK cells and subsets as well as on T CD4 and T CD8 lymphocytes and their ratio. A large sample of FA patients (n = 42) was used in this work, and the results were correlated to clinical hematological status of these patients. Among FA patients, a decreased proportion of T CD8(+) and NK CD56(dim)CD16(+) cells were observed when compared to healthy controls as well as an imbalance of the subsets NK lymphocytes. Data suggest that FA patients might have a defective cytotoxic response due to the lower number of cytotoxic cells as well as impairment in the differentiation process of the NK cells subsets which may be directly related to impairment of the immune surveillance observed in these patients.
Departamento de Bioquímica, IBRAG, UERJ, Rio de Janeiro, Brazil, magrajusto@hotmail.com.
Graça A
GA
Justo
Marco A
MA
Bitencourt
Ricardo
R
Pasquini
Morgana T L
MT
Castelo-Branco
Aline
A
Almeida-Oliveira
Hilda Rachel
HR
Diamond
Vivian M
VM
Rumjanek
D000293:Adolescent; D000328:Adult; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D019002:CD56 Antigen / Q000235:genetics / Q000276:immunology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D016022:Case-Control Studies; D002454:Cell Differentiation; D019070:Cell Lineage / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003602:Cytotoxicity, Immunologic; D005199:Fanconi Anemia / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D005260:Female; D058851:GPI-Linked Proteins / Q000235:genetics / Q000276:immunology; D015870:Gene Expression; D006801:Humans; D007157:Immunologic Surveillance; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D018655:Lymphocyte Count; D008297:Male; D017452:Receptors, IgG / Q000235:genetics / Q000276:immunology*
D019002:CD56 Antigen; C428200:FCGR3B protein, human; D058851:GPI-Linked Proteins; C522918:NCAM1 protein, human; D017452:Receptors, IgG