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Schwere Chronische Neutropenie Die schwere angeborene Neutropenie wird im 1. Lebensjahr symptomatisch mit schweren bakteriellen Infektionen, Pneumonien, Otitiden, Stomatitiden, Abszessen, Haut- und Schleimhautulzerationen. Antibiotikatherapie von Infektionen und –prophylaxe sind notwendig, subkutan verabreichter Granulozyten-Kolonie-stimulierender Faktor (G-CSF) führt zu einer subnormalen Neutrophilenzahl. 5-10% der Kinder zeigen aber kein Ansprechen auf G-CSF. Einzig kurative Therapie ist derzeit die Stammzell- bzw. Knochenmark-Transplantation (SCT/KMT). Unter Antibiotikaprophylaxe und G-CSF ist ein Erreichen des 2. Dezenniums beschrieben, ohne allogene SCT/KMT ist die Prognose aber infaust. Es besteht ein erhöhtes Risiko für die Entstehung einer akuten myeloischen Leukämie (ca. 10%). Stammzellen , Stammzellspender Leukämie Leukämiepatienten Zentrales Knochenmarkspender-Register Deutschland ,Dkms Typisierungsaktion in Berlin , HLA-A-, -B-Typisierung , Transplantation Morbus Kostmann und andere Formen der schweren angeborenen Neutropenie Schwachman-Diamond-Syndrom Zyklische Neutropenie Aplastische Anämie Myelodysplastisches Syndrom Retikuläre Dysgenesie Hyper-IgM-Syndrom Schwerer kombinierter Immundefekt (SCID) mit Graft-versus-Host-Disease Familiäre hämophagozytierende Lymphohistiozytose (FHL) Leukämie Lymphome und andere, das Knochenmark infiltrierende Malignomeretikuläre Dysgenesie Wiskott-Aldrich-Syndrom, Chediak-Higashi-Syndrom, Dyskeratosis congenita / Hoyeraal-Hreidarsson-Syndrom, Knorpel-Haar-Hypoplasie, Immuno-ossäre Dysplasie Schimke ,WHIM-Syndrom (Myelokathexis) Melisa Nidanur , Mirac Batuhan werden in Charite Virchow klinik behandelt. Spenden Aktion in Berlin Mitte, Neuköln, Kreuzberg, Reinickendorf, Schöneberg, Prenzlauerberg ,Spandau , berlinde Türkler kan bagschlaganfall
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