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Wiskott-Aldrich syndrome with unusual clinical features similar to juvenile myelomonocytic leukemia.

A male infant exhibited thrombocytopenia at birth, and later developed leukocytosis, monocytosis, and bloody stool. The bone marrow was hypercellular with dysplasia. Spontaneous granulocyte/macrophage-colony formation and hypersensitivity to granulocyte/macrophage-colony stimulating factor were confirmed by in vitro culture. These findings fulfilled most of the diagnostic criteria for juvenile myelomonocytic leukemia (JMML), with the exception of splenomegaly. However, no mutations in the PTPN11, RAS, and CBL genes, or clinical features of neurofibromatosis type 1, which are associated with JM

Human fibroblasts maintain the viability and augment the functional response of human neutrophils in culture.

When human neutrophils were co-cultured for 72 h with nontransformed human fibroblasts, 69 +/- 3% (n = 13) survived, as compared with survival levels of 2 +/- 1% (n = 15) and 26 +/- 6% (n = 7), respectively, for neutrophils cultured for the same time period in enriched medium alone or supplemented with 10 pM recombinant human granulocyte/macrophage colony-stimulating factor (rh GM-CSF). Conditioned medium from the human fibroblast cultures enhanced neutrophil survival in a dose-dependent fashion to the same level achieved with neutrophil/fibroblast co-cultures, and its soluble viability-sustai

Polyphenols from Lonicera caerulea L. berry attenuate experimental nonalcoholic steatohepatitis by inhibiting proinflammatory cytokines productions and lipid peroxidation.

SCOPE: Nonalcoholic steatohepatitis (NASH) is a common disease, which is closely associated with inflammation and oxidative stress, and Loni … histopathological features of NASH with lower levels of lipid peroxidation and cytokines including granulocyte colony-stimulating factor, IL-3, IL-4, macrophage inflammatory protein-1β, IL-6, IL-5, keratinocyte-derived cytokine, tumor necrosis factor-alpha, IL-2, IL-1β, monocytes chemotactic protein-1, IL-13, IFN-γ, IL-10, IL-12(p70), IL-1α, eotaxin, granulocyte-macrophage colony-sti …

Pseudomonas aeruginosa orbital phlegmon in a patient treated for myelodysplastic syndrome with concomitant Sjögren's syndrome.

Pseudomonas aeruginosa orbital infections have been described very rarely in patients with neutropenia after chemotherapy. We report the case of a woman with the unusual association of Sjögren's disease and myelodysplasia, who suffered from a Pseudomonas aeruginosa orbital phlegmon after chemotherapy for her myelodysplastic syndrome. Partial intestinal antibiotic decontamination with ciprofloxacine did not prevent the infection. She was treated successfully with intravenous ceftazidime, netilmicin and granulocyte-colony stimulating factor (G-CSF). The normalization of the granulocyte count see

Clinical toxicity of cytokines used as haemopoietic growth factors.

A number of cytokines are used as haemopoietic growth factors and this review focuses on toxicities associated with granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte colony-stimulating factor (G-CSF), interleukin (IL)-1, IL-3, IL-4, IL-6 and macrophage colony-stimulating factor (M-CSF). Both GM-CSF and G-CSF, currently approved for clinical use, are generally well tolerated by the majority of patients during short term administration. Constitutional symptoms and bone pain are the most frequently reported adverse effects, but they are rarely treatment-limiting. Reactivation…

Donor CMV serostatus has no impact on CMV viremia or disease when prophylactic granulocyte transfusions are given following allogeneic peripheral blood stem cell transplantation.

We studied the impact of donor cytomegalovirus (CMV) serologic status on CMV viremia and disease when prophylactic granulocyte colony-stimulating factor (G-CSF)-mobilized granulocyte transfusions (GTs) were given following allogeneic peripheral blood stem cell (AlloPBSC) transplantation. A cohort of 83 patients who received 2 prophylactic GTs from ABO-compatible stem cell donors following AlloPBSC transplantation was compared with a cohort of 142 patients who did not. AlloPBSC donors were eligible for granulocyte donation irrespective of their CMV serostatus. Recipients received no prophylacti…

Hereditary pulmonary alveolar proteinosis. Could it be triggered by Mycoplasma pneumoniae pneumonia?

We present a three-year-old girl with respiratory failure due to hereditary pulmonary alveolar proteinosis caused by abnormal alpha chain of the granulocyte-macrophage colony-stimulating factor receptor. Both the patient and an asymptomatic seven-year-old sister were homozygous for the same mutation in CSF2RA. We speculate that the Mycoplasma pneumoniae pneumonia might have triggered the clinical presentation. While a good response to serial partial lung lavage was noticed, the ultimate outcome is uncertain.

Successful reduced-intensity stem cell transplantation in a patient with myelodysplastic syndrome combined with Sweet's syndrome.

A 53-year-old male with myelodysplastic syndrome developed Sweet's syndrome extensively over his left iliac and inguinal regions that was refractory to standard treatment with corticosteroids and chemotherapy, received a stem cell transplant from an HLA-matched unrelated donor, conditioned by reduced-intensity regimen. The patient achieved complete hematological remission, and the cutaneous lesions improved gradually and then disappeared completely despite the patient receiving granulocyte colony-stimulating factor after transplantation and developing acute graft-versus-host disease.

Reversible agranulocytosis associated with oral terbinafine in a pediatric patient.

We report a case of terbinafine-induced agranulocytosis in a pediatric patient. This effect was noted 4 weeks after starting terbinafine and resolved within 1 week without the institution of therapy with granulocyte colony-stimulating factor, a course similar to reports in adults. With the incidence of severe side effects still unclear in children, this case argues for continued blood surveillance during courses of terbinafine and for the confirmation of infection before initiation of the drug.

Clozapine-induced agranulocytosis and use of g-csf.

Use of clozapine is attended with the serious though rare risk of agranulocytosis. Clozapineinduced agranulocytosis is reversible with the use of cytokines like granulocyte-colony stimulating factor (G-CSF). Reports of the haematological complication of clozapine have not been forthcoming from India though it has been in use for nearly three years. This report is on an young patient who developed total absence of granulocytes during the 4th month of treatment who was successfully treated with G-CSF.

Long-term remission of neutropenia in Felty's syndrome after a short GM-CSF treatment.

We report a case of Felty's syndrome in which neutropenia was corrected by a short-term treatment with recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF, 5 micrograms/kg/day s.c. for 14 days). Absolute neutrophil counts rose from 0.1 to 2.2 x 10(9)/l and remained > 1.0 x 10(9)/l 8 weeks after discontinuation of the GM-CSF therapy. A flare-up of arthritis and a decrease in platelet counts were observed.

Clinical use of GM-CSF in autologous bone marrow transplantation.

Over the last decade, the key role of specific glycoproteins (hematopoietic growth factors) in the proliferation and maturation of hematopoietic cells (in conjunction with some of their particular functional activities) has been demonstrated through in vitro and in vivo studies. These glycoproteins have been evaluated for their potential application in reducing the duration of myelosuppression following conventional chemotherapy and autologous bone marrow transplantation. Preliminary results using granulocyte-macrophage colony-stimulating factor to restore neutrophil competence seem to be enco

Interleukin-2 treatment-associated eosinophilia is mediated by interleukin-5 production.

During a trial using recombinant human interleukin-2 (rhIL-2) immunotherapy for acute myeloblastic leukaemia (AML) in remission, eosinophilia was observed in all patients. We used in-vitro clonogenic assays to investigate the mechanism of the eosinophilia in five patients. The mean eosinophil count increased from 0.05 x 10(9)/l before rhIL-2 to 0.98 x 10(9)/l within 48 h of stopping the infusion, and an exponential correlation between the pretreatment lymphocyte CD4:CD8 ratio and the maximum eosinophil count was observed. RhIL-2 did not stimulate eosinophil colony formation by normal bone marr

Только G-CSF + плериксафор против G-CSF мобилизовали гематопоиетические стволовые клетки у пациентов с множественной миеломой и лимфомой: систематический обзор и метаанализ.

Комбинация стимулирующих факторов гранулоцит-колония (G-CSF) и плериксафор: one Подходы к гематопоиетической мобилизации стволовых клеток у пациентов с множественной миеломой (ММ), лимфомой не Ходжкина (НХЛ) и лимфомой Ходжкина (ХЛ). Цель этого систематического обзора и метаанализа состояла в том, чтобы определить способность G-CSF + Prixafor мобилизовать периферийные клетки крови (PB) CD34+ и изучить свой профиль безопасности. Methods Мы провели поиск в базе данных с использованием терминов "гранулоцитная колония", "G-CSF", "AMD3100" и "plicxafor", опубликованных до мая. 1, 2023. Более подроб

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… G-CSF, granulocyte colony-stimulating factor; HL, Hodgkin’s lymphoma; MM, multiple myeloma; NA, not available; NHL, non-Hodgkin’s lymphoma. … Phase III prospective randomized Double-Blind Placebo-Controlled trial of plerixafor plus granulocyte colony-stimulating factor compared with placebo plus granulocyte colony-stimulating factor for autologous stem-cell mobilization and transplantation for patients with non-Hodgkin’s lymphoma. …

Clinical, immunological and microbial gingival profile of juvenile systemic lupus erythematosus patients.

Periodontal disease has been associated with rheumatic diseases; however, few studies have evaluated the association with systemic lupus ery … ttachment loss. After adjustment for multiple comparisons, GCF levels of interleukin (IL)-1β, IL-8, granulocyte colony-stimulating factor (G-CSF), interferon-γ and monocyte chemoattractant protein-1 were significantly higher, whereas the levels of granulocyte-macrophage colony-stimulating factor were significantly lower in jSLE patients. In serum, G-CSF levels tended to be higher in j …

Serum and follicular fluid monocyte chemotactic protein-1 levels are elevated in obese women and are associated with poorer clinical pregnancy rate after in vitro fertilization: a pilot study.

OBJECTIVE: To determine whether monocyte chemotactic protein-1 (MCP-1), a proinflammatory chemokine important in ovulation, is abnormally elevated in obese women undergoing IVF and whether serum and follicular fluid (FF) levels of MCP-1 are associated with IVF outcome. DESIGN: Prospective pilot study. SETTING: Academic center. PATIENT(S): Women undergoing IVF. INTERVENTION(S): Serum and FF were collected from women undergoing IVF. MAIN OUTCOME MEASURE(S): Correlation between MCP-1 and other inflammatory markers with adiposity and pregnancy outcome after IVF. RESULT(S): Obese women had si

The proliferative effects of asbestos-exposed peripheral blood mononuclear cells on mesothelial cells.

Malignant mesothelioma (MM) is thought to arise from the direct effect of asbestos on mesothelial cells. However, MM takes a long time to de … PBMC culture exposed to CA or CR. Analysis of cytokine production demonstrated increased levels of granulocyte colony-stimulating factor (G-CSF), granulocyte-macrophage colony-stimulating factor (GM-CSF), interleukin (IL)-1α, IL-1β, IL-3, IL-5, IL-13 and IL-17A in supernatants. Individual administration of these cytokines, excluding G-CSF and GM-CSF, led to an increase in cell growth …

Bone marrow-derived cells promote liver regeneration in mice with erythropoietic protoporphyria.

BACKGROUND: Bone marrow transplantation can reverse hepatic protoporphyrin accumulation and prevent the hepatobiliary complications characte … uring 12 or 36 weeks. Two groups of animals killed 12 weeks after transplant were also treated with granulocyte colony-stimulating factor. RESULTS: Cell transplantation decreased porphyrin contents in erythrocytes and liver. Improved hepatic structure and function and reduced hepatic fibrosis were observed, especially 36 weeks after transplant. Bone marrow-derived cells (22%-35%) wer …

Neutrophil biology and the next generation of myeloid growth factors.

Neutrophils are the body's critical phagocytic cells for defense against bacterial and fungal infections; bone marrow must produce approximately 10 x 10(9) neutrophils/kg/d to maintain normal blood neutrophil counts. Production of neutrophils depends on myeloid growth factors, particularly granulocyte colony-stimulating factor (G-CSF). After the original phase of development, researchers modified these growth factors to increase their size and delay renal clearance, increase their biologic potency, and create unique molecules for business purposes. Pegylated G-CSF is a successful product of th…

Cytokine-induced transcriptional changes in human neutrophils reveal immune regulatory plasticity.

INTRODUCTION: Neutrophils are innate immune cells that play a central role in the inflammatory response. They produce an array of destructiv … s that can drive the differentiation and activation of CD4 T-cells. Stimulation of neutrophils with granulocyte colony-stimulating factor, interferon alpha, interferon gamma, interleukin-1 beta, or interleukin-8 resulted in expression of discrete gene sets and differential activation of signalling pathways including changes in cell adhesion and migration, immune receptor expression, a …