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Titolo:
Severe congenital neuropenia: Diagnosis and therapy
Autore:
Zeidler, C; Schwinzer, B; Welte, K;
Indirizzi:
Med Hsch Hannover, Kinderklin, Datencoordinat Zentrum Europa, SCNIR, D-30623 Hannover, Germany Med Hsch Hannover Hannover Germany D-30623 IR, D-30623 Hannover, Germany
Titolo Testata:
KLINISCHE PADIATRIE
fascicolo: 4, volume: 212, anno: 2000,
pagine: 145 - 152
SICI:
0300-8630(200007/08)212:4<145:SCNDAT>2.0.ZU;2-X
Fonte:
ISI
Lingua:
GER
Soggetto:
COLONY-STIMULATING-FACTOR; SEVERE CHRONIC NEUTROPENIA; ACUTE MYELOID-LEUKEMIA; CYCLIC NEUTROPENIA; SHWACHMAN-SYNDROME; KOSTMANNS-SYNDROME; FACTOR-RECEPTOR; GRANULOCYTE; AGRANULOCYTOSIS; NEUTROPHILS;
Keywords:
neutropenia; G-CSF-diagnosis; therapy;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
44
Recensione:
Indirizzi per estratti:
Indirizzo: Zeidler, C Med Hsch Hannover, Kinderklin, Datencoordinat Zentrum Europa, SCNIR, D-30623 Hannover, Germany Med Hsch Hannover Hannover Germany D-30623 Hannover, Germany
Citazione:
C. Zeidler et al., "Severe congenital neuropenia: Diagnosis and therapy", KLIN PADIAT, 212(4), 2000, pp. 145-152

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 with absolute neutrophil counts (ANC) less than 0.2 x 10(9)/l associatedwith severe systemic bacterial infections from early infancy. Data on over300 patients with CN collected by the Severe Chronic Neutropenia International Registry (SCNIR) since 1994 indicate that >90% of these patients respond to recombinant human granulocyte-colony stimulating factor (rHuG-CSF) treatment with an ANC > 1.0 x 10(9)/l. In these patients rHuG-CSF is requireddaily as subcutaneous injection with individual doses ranging between 0.27and 120 mcg/kg/day to maintain ANC above 1.0 x 10(9)/l. Adverse events documented in this group of patients include splenomegaly, thrombocytopenia, osteoporosis and malignant transformation into MDS/leukemia. If and how rHuG-CSF treatment impacts on these adverse events remains unclear since there are no historical controls for comparison. For those patients who are refractory to rHuG-CSF treatment and continue to have severe and often life-threatening bacterial infections, hematopoietic stem cell transplantation (HSCT) is still the only currently available treatment.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/04/20 alle ore 07:56:12