Rapid cell senescence and apoptosis in lymphocytes and granulocytes and absence of GM-CSF receptor in congenital dysgranulopoietic neutropenia.
A girl with congenital dysgranulopoietic neutropenia (CDN) and her non-neutropenic mother with aphthae (A) were investigated. Apoptosis in lymphocytes and granulocytes of both patients (mother A+) were documented by high annexin and electron microscopic morphology. CD11b/CD18 of the daughter's granulocytes ranged between low to normal while that of the mother changed between very low to high levels through A(-) to A(+) periods. In both patients, CD11b/CD18 on lymphocytes were high; GM-CSF receptor was negative; CD4-/CD8- lymphocytes were high and the leukocytes which showed abnormal cell cycle were stained by senescence associated beta-galactosidase. We think that increased apoptosis and rapid cell senescence of leukocytes underlies the pathophysiology of CDN.
Olcay
Lale
L
Dr AY Ankara Oncology Research and Education Hospital, Unit of Pediatric Hematology, Demetevler, Ankara, Turkey. laleolcay@hotmail.com.tr
Yetgin
Sevgi
S
Okur
Hamza
H
Erdemli
Esra
E
D000293:Adolescent; D017304:Annexin A5 / Q000378:metabolism; D015703:Antigens, CD / Q000378:metabolism; D017209:Apoptosis / Q000502:physiology*; D002453:Cell Cycle; D016922:Cellular Senescence / Q000502:physiology*; D005260:Female; D005434:Flow Cytometry; D005455:Fluorescent Antibody Technique; D006098:Granulocytes / Q000648:ultrastructure*; D006801:Humans; D016131:Lymphocyte Subsets / Q000648:ultrastructure; D008214:Lymphocytes / Q000648:ultrastructure*; D046529:Microscopy, Electron, Transmission; D009503:Neutropenia / Q000151:congenital / Q000473:pathology / Q000503:physiopathology*; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism; D016187:Receptors, Granulocyte-Macrophage Colony-Stimulating Factor / Q000172:deficiency*; D013281:Stomatitis, Aphthous / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D019014:fas Receptor / Q000378:metabolism
D017304:Annexin A5; D015703:Antigens, CD; D019253:Proto-Oncogene Proteins c-bcl-2; D016187:Receptors, Granulocyte-Macrophage Colony-Stimulating Factor; D019014:fas Receptor