Rapid cell senescence and apoptosis in lymphocytes and granulocytes and absence of GM-CSF receptor in congenital dysgranulopoietic neutropenia


Olcay L., YETGİN S. , OKUR H. , ERDEMLİ E.

LEUKEMIA RESEARCH, cilt.32, ss.235-242, 2008 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 32 Konu: 2
  • Basım Tarihi: 2008
  • Doi Numarası: 10.1016/j.leukres.2007.06.017
  • Dergi Adı: LEUKEMIA RESEARCH
  • Sayfa Sayıları: ss.235-242

Özet

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. (C) 2007 Elsevier Ltd. All rights reserved.