Treosulfan-based conditioning regimen in a second matched unrelated peripheral blood stem cell transplantation for a pediatric patient with CGD and invasive aspergillosis, who experienced initial graft failure after RIC.

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by a defect of phagocyte NADPH-oxidase and characterized by severe, recurrent bacterial and fungal infections. Invasive aspergillosis (IA) is the leading cause of mortality in patients with CGD. We report the case of a 3-year-old boy with CGD, who developed IA despite antifungal prophylaxis.

Chronic granulomatous disease: the European experience.

CGD is an immunodeficiency caused by deletions or mutations in genes that encode subunits of the leukocyte NADPH oxidase complex. Normally, assembly of the NADPH oxidase complex in phagosomes of certain phagocytic cells leads to a "respiratory burst", essential for the clearance of phagocytosed micro-organisms. CGD patients lack this mechanism, which leads to life-threatening infections and granuloma formation.

[Difficulties in diagnostics and therapy of infectious complications in primary immunodeficient patients].

Primary immunodeficiencies (PIDs) are group of more than 200 different genetic disorders. Reccurrent, severe infections are major clinical manifestation of these disorders. Ethiology and course of infections in PIDs are different, depending on type of immunodeficiency.

[Chronic neutropenia - experience from the Department of Immunology, Children's Memorial Health Institute].

Unlabelled: Chronic neutropenia (CN) is defined by an absolute neutrophil count (ANC) below 1500/ul, lasting at least 6 months.

Aim: clinical course and treatment of children afflicted with CN was analysed.

Material And Methods: we present 60 children treated in our department due to CN.