Expression of receptors for granulocyte colony-stimulating factor on neutrophils from patients with severe congenital neutropenia and cyclic neutropenia

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Expression of receptors for granulocyte colony-stimulating factor on neutrophils from patients with severe congenital neutropenia and cyclic neutropenia.

We studied granulocyte colony-stimulating factor (G-CSF) binding sites on neutrophils from patients with severe congenital neutropenia (SCN; Kostmann-syndrome) and cyclic neutropenia (CN) during treatment with recombinant human (rh) G-CSF. G-CSF receptor expression was measured by scatchard analysis. Neutrophils from six healthy controls expressed between 480 and 1,210 binding sites per cell, w...

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Differential effects of granulocyte-macrophage colony-stimulating factor and granulocyte colony-stimulating factor in children with severe congenital neutropenia.

Severe congenital neutropenia (SCN) is a disorder of myelopoiesis characterized by severe neutropenia secondary to a maturational arrest at the level of promyelocytes. We treated five patients with SCN with recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) for 42 days and subsequently, between 1 and 3 months later, with rhG-CSF for 142 days. The objective was to eval...

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Increased serum levels of granulocyte colony-stimulating factor in patients with severe congenital neutropenia.

Severe congenital neutropenia (SCN), also known as Kostmann Syndrome, is characterized by a maturation arrest of myelopoiesis at the level of promyelocytes with absence of neutrophils in bone marrow (BM) and blood. Hypotheses of the pathophysiology of SCN include (1) defective production of granulocyte colony-stimulating factor (G-CSF), and/or (2) defective response to G-CSF. To exclude defecti...

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Spontaneous remission of granulocyte colony-stimulating factor-associated leukemia in a child with severe congenital neutropenia.

Leukemia is observed with increased frequency in patients with severe congenital neutropenia (SCN). In the past decade, recombinant human granulocyte colony-stimulating factor (rh G-CSF) has prolonged the survival of patients with SCN increasingly reported to have leukemias. In this communication acute myelogenous leukemia (AML) associated with a mutation of the G-CSF receptor (G-CSF-R) develop...

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The Effect of Granulocyte Colony Stimulating Factor Administration on Preterm Infant with Neutropenia and Clinical Sepsis: A Randomized Clinical Trial

Abstract Background This study was conducted to evaluate the clinical effect of Granulocyte Colony Stimulating Factor (GCSF) on prognosis of neonatal sepsis. Materials and Methods Present study is a double- blinded randomized clinical trial, conducted on 46 preterm infants with neutropenia (Absolute Neutrophil Count (ANC) ≤ 5000 / μL) and clinical sepsis. Infants were randomly allocated ...

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ژورنال

عنوان ژورنال: Blood

سال: 1992

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v79.5.1144.1144