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Titolo:
Alkaline phosphatase activity in neutrophils from patients with severe congenital neutropenia (Kostmann's syndrome)
Autore:
Baranova, K; Stanulla, M; Zeidler, C; Welte, K;
Indirizzi:
Hannover Med Sch, Dept Pediat Hematol & Oncol, Childrens Hosp, D-30625 Hannover, Germany Hannover Med Sch Hannover Germany D-30625 osp, D-30625 Hannover, Germany Gomel Med Ctr, Gomel, Byelarus Gomel Med Ctr Gomel ByelarusGomel Med Ctr, Gomel, Byelarus
Titolo Testata:
INTERNATIONAL JOURNAL OF HEMATOLOGY
fascicolo: 4, volume: 70, anno: 1999,
pagine: 236 - 240
SICI:
0925-5710(199912)70:4<236:APAINF>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
COLONY-STIMULATING FACTOR; MESSENGER-RNA; NORMAL INDIVIDUALS; GRANULOCYTES; EXPRESSION; LEUKEMIA; DISEASE; INVITRO; CELLS; CSF;
Keywords:
agranulocytosis; alkaline phosphatase; congenital neutropenia; granulocyte colony-stimulating factor; Kostmann's syndrome; neutrophils;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
25
Recensione:
Indirizzi per estratti:
Indirizzo: Welte, K Hannover Med Sch, Dept Pediat Hematol & Oncol, Childrens Hosp, Carl Neuberg Str 1, D-30625 Hannover, Germany Hannover Med Sch Carl Neuberg Str 1 Hannover Germany D-30625 any
Citazione:
K. Baranova et al., "Alkaline phosphatase activity in neutrophils from patients with severe congenital neutropenia (Kostmann's syndrome)", INT J HEMAT, 70(4), 1999, pp. 236-240

Abstract

The glycoprotein alkaline leukocyte phosphatase (ALP) can be used as a marker of maturity in neutrophilic granulocytes as the activity of ALP increases during neutrophilic differentiation, Severe congenital neutropenia (SCN)or Kostmann's syndrome is a congenital disorder characterized by a maturation arrest of myeloid progenitor cells at the promyelocyte/myelocyte stage that can be treated with recombinant human granulocyte colony-stimulating factor (rhG-CSF). Until recently there have been no reports on ALP activity in neutrophils of patients suffering from SCN, despite the fact that ALP isan important correlate of the functional activity of normal neutrophils. Thus. we conducted experiments to assess ALP activity in neutrophils from eight SCN patients before initiation of rhG-CSF treatment and an additional 17 SCN patients already receiving rhG-CSF. All eight patients analyzed before initiation of rhG-CSF treatment showed ALP activity in their neutrophiliccells. Four patients had normal and four patients had elevated ALP levels. Of the 17 patients already on treatment with rhG-CSE 15 patients showed elevated ALP activity levels, one patient had normal ALP levels, and one patient showed abnormally low ALP activity. Thus with the exception of a singlepatient, we observed normal or elevated ALP activity in neutrophils from SCN patients. As described in studies on the effect of rhG-CSF on ALP activity in healthy individuals, ALP activity was higher in SCN patients already on treatment with rhG-CSF. Therefore, our results indicate that SCN patients are not deficient in ALP activity and suggest that ALP is not deregulatedin the majority of these patients. (C) 1999 The Japanese Society of Hematology.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/04/20 alle ore 17:58:20