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.
Dr AY Ankara Oncology Research and Education Hospital, Unit of Pediatric Hematology, Demetevler, Ankara, Turkey. laleolcay@hotmail.com.tr
Lale
L
Olcay
Sevgi
S
Yetgin
Hamza
H
Okur
Esra
E
Erdemli
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