Combined corticosteroid/granulocyte colony-stimulating factor (G-CSF) therapy in the treatment of severe congenital neutropenia unresponsive to G-CSF

Dror, Yigal; Ward, Alister C.; Touw, Ivo P.; Freedman, Melvin H.

doi:10.1016/s0301-472x(00)00544-0

Cited by 37 publications

(32 citation statements)

References 53 publications

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“…Interestingly, a clinical study of a child with SCN who was unresponsive to G-CSF and harboured a mutation in the extracellular domain of the G-CSFR, as described previously by Ward et al [150], showed that small daily doses of oral prednisone administered with conventional doses of G-CSF, restored the patient' response, as evidenced by increased neutrophil numbers and reversal of infections [153]. The combination of G-CSF and dexamethasone also improved proliferation and STAT5 activation of myeloid cells expressing the mutated receptor.…”

Section: Identification Of Mutations and Polymorphisms In G-csfrmentioning

confidence: 72%

“…Reduced activation of STAT5, unresponsive to G-CSF [150,153] 182-bp deletion in the region coding for the extracellular domain Truncation in the extracellular portion of the G-CSFR Lack of response to pharmacologic doses of filgrastim [151] 319 mutation in the region coding within the WSXWS motif in the extracellular portion Constitutive JAK2 activity in vitro [169] C > A point mutation at nucleotide 2624…”

Section: G-csfrmentioning

confidence: 99%

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Pharmacologic rationale for early G-CSF prophylaxis in cancer patients and role of pharmacogenetics in treatment optimization

Crea

Giovannetti

Zinzani

et al. 2009

Critical Reviews in Oncology/Hematology

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Section: Identification Of Mutations and Polymorphisms In G-csfrmentioning

confidence: 72%

Section: G-csfrmentioning

confidence: 99%

Pharmacologic rationale for early G-CSF prophylaxis in cancer patients and role of pharmacogenetics in treatment optimization

Crea

Giovannetti

Zinzani

et al. 2009

Critical Reviews in Oncology/Hematology

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“…In vitro studies have confirmed that cortisol and synthetic glucocorticoids were able to inhibit neutrophil apoptosis (Weyts et al ., 1998;Zhang et al ., 2001) and this is likely to underlie the extension of neutrophil lifespan in the human in vivo studies. Cortisol has also been shown to enhance the induction of neutrophil production from haemopoietic stem cells by granulocyte-colony stimulating factor (G-CSF) (Dror et al ., 2000), giving an additional mechanism for its effects on circulating neutrophil numbers. Taken together, these effects of stress hormones would be expected to improve neutrophil-mediated immunity.…”

Section: Glucocorticoids and Immunitymentioning

confidence: 99%

Stress responses and innate immunity: aging as a contributory factor

2004

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SummaryEvolutionary pressure has selected individuals with traits that allow them to survive to reproduction, without consideration of the consequences for the post-child rearing years and old age. In the 21st century, society is populated increasingly by the elderly and with the falling birth rate and improved health care this trend is set to continue for the foreseeable future. To minimize the potential burden on health services one would hope that 'growing old gracefully' should also mean 'growing old healthily'. However, for too many the aging process is accompanied by increasing physical and mental frailty producing an elevated risk of physical and psychological stress in old age. Stress is a potent modulator of immune function, which in youth can be compensated for by the presence of an optimal immune response. In the elderly the immune response is blunted as a result of the decline in several components of the immune system (immune senescence) and a shifting to a chronic pro-inflammatory status (the so-called 'inflammaging' effect). We discuss here what is known of the effects of both stress and aging upon the innate immune system, focusing in particular upon the age-related alterations in the hypopituitary-adrenal axis. We propose a double hit model for age and stress in which the age-related increase in the cortisol/sulphated dehydroepiandrosterone ratio synergizes with elevated cortisol during stress to reduce immunity in the elderly significantly.

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“…Combination therapy with G-CSF and prednisone was found to be effective in treating the P206H patient, 60 whereas GM-CSF alone or G-CSF in combination with SCF proved ineffective. Our patient, like the ⌬322 patient, failed to respond to high-dose G-CSF, or other cytokines, and was successfully treated with a stem cell transplantation.…”

Section: Discussionmentioning

confidence: 99%

Novel mechanism of G-CSF refractoriness in patients with severe congenital neutropenia

Druhan

Massullo

et al. 2005

Blood

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Severe congenital neutropenia (SCN) is a rare disease diagnosed at or soon after birth, characterized by a myeloid maturation arrest in the bone marrow, ineffective neutrophil production, and recurrent infections. Most patients respond to treatment with granulocyte colony-stimulating factor (G-CSF), and the majority harbor mutations in the neutrophil elastase gene. In the subset of patients with SCN transforming to acute myeloid leukemia (

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Combined corticosteroid/granulocyte colony-stimulating factor (G-CSF) therapy in the treatment of severe congenital neutropenia unresponsive to G-CSF

Cited by 37 publications

References 53 publications

Pharmacologic rationale for early G-CSF prophylaxis in cancer patients and role of pharmacogenetics in treatment optimization

Pharmacologic rationale for early G-CSF prophylaxis in cancer patients and role of pharmacogenetics in treatment optimization

Stress responses and innate immunity: aging as a contributory factor

Novel mechanism of G-CSF refractoriness in patients with severe congenital neutropenia

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