Burst test using FagoFlow in patients with CGD and hematopeotic stem cell transplantation Andrea Poloučková, M.D. Department of Immunology, Charles University, 2nd Medical Faculty and University Hospital Motol, Prague Phagocytosis Is Always the Best Time of Day
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Burst test using FagoFlow in patients with CGD and hematopeotic stem cell transplantation Andrea Poloučková, M.D. Department of Immunology, Charles University,
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Burst test using FagoFlow in patients with CGD and hematopeotic stem cell transplantation
Andrea Poloučková, M.D.Department of Immunology, Charles University, 2nd Medical Faculty and University Hospital Motol, Prague
Phagocytosis Is Always the Best Time of Day
CGD diagnosis
• NBT (nitro-blue tetrazolium chlorid)
• Chemiluminiscence• Flow cytometry – Fago Flow test is based on the
measurement of respiratory (oxidative) burst of granulocytes after their stimulation with E. coli bacteria. After the ingestion of bacteria, phagocytes activate the NADP oxidase producing reactive oxidative intermediates (respiratory burst). Reactive intermediates inside phagocytes oxidize dihydrorhodamine 123 (DHR123) into fluorescent rhodamine123 which is detected by a flow cytometer
• Enzyme activity (MPO)
• Molecular - genetic analysis of the defect genes
CGD therapy• Antibiotic and antifungal profylaxis and
therapy
• Neutrophil infusions
• Ɣ-INF
• Hematopoetic stem cell transplantation – Goudemand J, Anssens R, Delmas-Marsalet Y, Farriaux JP, Fontaine G: [Attempt to treat
a case of chronic familial granulomatous disease by allogenic bone marrow transplantation]. Arch Fr Pediatr 1976, 33(2):121-129.
– HLA identical family donor, complicated course of the disease
• Gene therapy– Ott, M. G. et al. Correction of X-linked chronic granulomatous disease by gene therapy,
augmented by insertional activation of MDS1-EVI1, PRDM16 or SETBP1. Nature Med. 2 Apr 2006
Patient 1(PB, 2006)
• FH:negative
• from 9 months – respiratory infections
• 18 months - multiple liver foci – susp. on malignancy, transfer to our hospital
• without IS treatment, no signs of GVHD• permanent increase of the mixed chimerism (D+70 29% autolougous chimerism, 1 year after Tx 75% autologous chimerism)• decrease in NBT and FagoFlow • NBT test: 12 - 7 – 6 - 4 (normal range > 9)• FagoFlow test: 65% - 77% - 22% (normal range > 76)• without clinical problems• therapy: penicilin