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Titolo:
Stem cell transplantation in patients with severe congenital neutropenia without evidence of leukemic transformation
Autore:
Zeidler, C; Welte, K; Barak, Y; Barriga, F; Bolyard, AA; Boxer, L; Cornu, G; Cowan, MJ; Dale, DC; Flood, T; Freedman, M; Gadner, H; Mandel, H; OReilly, RJ; Ramenghi, U; Reiter, A; Skinner, R; Vermylen, C; Levine, JE;
Indirizzi:
Med Hsch Hannover, Dept Pediat Hematol, Oncol Kinderklin, D-30625 Hannover, Germany Med Hsch Hannover Hannover Germany D-30625 in, D-30625 Hannover, Germany Kaplan Med Ctr, Rehovot, Israel Kaplan Med Ctr Rehovot IsraelKaplan Med Ctr, Rehovot, Israel Catholic Univ Chile, Santiago, Chile Catholic Univ Chile Santiago ChileCatholic Univ Chile, Santiago, Chile Univ Washington, Seattle, WA 98195 USA Univ Washington Seattle WA USA 98195 iv Washington, Seattle, WA 98195 USA Univ Michigan, Ann Arbor, MI 48109 USA Univ Michigan Ann Arbor MI USA 48109 iv Michigan, Ann Arbor, MI 48109 USA Catholic Univ Louvain, Clin Univ St Luc, Brussels, Belgium Catholic Univ Louvain Brussels Belgium n Univ St Luc, Brussels, Belgium Univ Calif San Francisco, San Francisco, CA 94143 USA Univ Calif San Francisco San Francisco CA USA 94143 ancisco, CA 94143 USA Sir James Spence Inst Child Hlth, Newcastle Upon Tyne, Tyne & Wear, England Sir James Spence Inst Child Hlth Newcastle Upon Tyne Tyne & Wear England Hosp Sick Children, Toronto, ON M5G 1X8, Canada Hosp Sick Children Toronto ON Canada M5G 1X8 Toronto, ON M5G 1X8, Canada St Anna Kinderspital, Vienna, Austria St Anna Kinderspital Vienna Austria Anna Kinderspital, Vienna, Austria Rambam Med Ctr, Haifa, Israel Rambam Med Ctr Haifa IsraelRambam Med Ctr, Haifa, Israel Mem Sloan Kettering Canc Ctr, New York, NY 10021 USA Mem Sloan Kettering Canc Ctr New York NY USA 10021 New York, NY 10021 USA Univ Turin, Dipartimento Sci Pediat, Turin, Italy Univ Turin Turin Italy niv Turin, Dipartimento Sci Pediat, Turin, Italy
Titolo Testata:
BLOOD
fascicolo: 4, volume: 95, anno: 2000,
pagine: 1195 - 1198
SICI:
0006-4971(20000215)95:4<1195:SCTIPW>2.0.ZU;2-U
Fonte:
ISI
Lingua:
ENG
Soggetto:
COLONY-STIMULATING-FACTOR; FACTOR-RECEPTOR GENE; R-METHUG-CSF; POINT MUTATIONS; GRANULOCYTE; AGRANULOCYTOSIS; PATHOPHYSIOLOGY; FILGRASTIM; THERAPY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
26
Recensione:
Indirizzi per estratti:
Indirizzo: Zeidler, C Med Hsch Hannover, Dept Pediat Hematol, Oncol Kinderklin, Carl Neuberg Str1, D-30625 Hannover, Germany Med Hsch Hannover Carl Neuberg Str 1 Hannover Germany D-30625
Citazione:
C. Zeidler et al., "Stem cell transplantation in patients with severe congenital neutropenia without evidence of leukemic transformation", BLOOD, 95(4), 2000, pp. 1195-1198

Abstract

Severe congenital neutropenia (CN) (Kostmann syndrome) is a hematologic disorder characterized by a maturation arrest of myelopoiesis at the promyelocyte/myelocyte stage of development, This arrest results in severe neutropenia leading to absolute neutrophil counts (ANC) below 0.2 x 10(9)/L associated with severe bacterial infections from early infancy. Data on over 300 patients with CN collected by the Severe Chronic Neutropenia International Registry (SCNIR) beginning in 1994 indicate that more than 90% of these patients respond to recombinant human granulocyte colony stimulating factor (r-HuG-CSF) treatment with an ANC greater than 1.0 x 10(9)/L. For patients whoare refractory to r-HUG-CSF treatment and continue to have severe and often life-threatening bacterial infections, hematopoietic stem cell transplantation is the only currently available treatment. We report on a total of 11patients with CN reported to the SCNIR who underwent transplantation for reasons other than malignant transformation between 1976 and 1998. Of these patients, 8 were nonresponders or showed only partial response to r-HuG-CSFtreatment with ongoing infections. Results from these patients suggest that transplantation of stem cells from an HLA-identical sibling is beneficialfor patients refractory to r-HuG-CSF. (Blood. 2000;95:1195-1198) (C) 2000 by The American Society of Hematology.

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Documento generato il 01/04/20 alle ore 01:54:26