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Bestimmte Mutationen im Bereich der a- beziehungsweise b-Ketten des Hämoglobins können zu Hochaffinitäts-Hämoglobinen führen, die Sauerstoff im peripheren Gewebe in verringertem Maße freisetzen. So liegt bei einem O2-Partialdruck von 20 mm Hg im Kapillarbereich 35 % Oxyhämoglobin vor, wohingegen zum Beispiel beim Hochaffinitäts-Hämoglobin Johnstown noch 60 % oxygeniert sind (Grafik 1). Die dadurch verursachte Verringerung der Sauerstoffabgabe im peripheren Gewebe führt zu einer kompensatorischen Erhöhung der Hämoglobin-Konzentration. Daneben kann auch eine Erniedrigung des intraerythrozytären 2,3-Bisphosphoglyceratspiegels durch 2,3-Bisposphoglyceratmutase-Mangel zu einer erhöhten Sauerstoffaffinität des Hämoglobins führen.
Der Blutdruck sank in den Normbereich, ebenso sanken der Hämatokrit und das Hämoglobin. Hier wird gewichtsadaptiert ein Aderlass durchgeführt und mit isotonischer Kochsalzlösung substituiert. Bei einer absoluten Polyglobulie erfolgt die weitere diagnostische Abklärung der Grunderkrankung. Dazu zählen unter anderem die Kardiodiagnostik mittels Echokardiographie, der Röntgenthorax, die Sonographie und ggf. Durch hohe Flüssigkeitsverluste nach lang andauerndem Erbrechen oder massiven Diarrhoen ändert sich das Zell-Plasma-Verhältnis.
Männer mit einer niedrigen Testosteronkonzentration sterben häufiger an Tumor- oder Herz-Kreislauf-Erkrankungen als Männer mit einer physiologischen Konzentration dieses Sexualhormons. So ist ein niedriger Testosteronspiegel häufig mit einem erhöhten allgemeinen Krankheitsrisiko assoziiert. Dies wurde in einer Studie mit 930 Männern mit koronarer Herzkrankheit (KHK) bestätigt. Über einen Zeitraum von sieben Jahren wurden alle Todesfälle erfasst und mit dem https://jcrunch.com/employer/wie-viel-ist-testosteron-verschreibungspflichtig/wert korreliert. In der Gruppe mit Hormondefizit warfare die Mortalitätsrate fast doppelt so hoch wie in der Gruppe mit normalen Testosteronspiegeln.
Das mit den Erythrozyten entfernte Eisen führt zu einem sekundären Eisenmangel, der die Erythropoese limitiert und zu Müdigkeit und Abnahme der Leistungsfähigkeit führen kann. Zunächst im Osten Russlands und später auch in Mitteleuropa fand man eine besondere Form der erblichen Polyzythämie. Ihr liegt eine autosomal-rezessiv vererbte Mutation im Von-Hippel-Lindau(VHL)-Gen zugrunde. Das VHL-Protein reguliert in den peritubulären Fibroblasten der Niere den Abbau des Hypoxie-induzierbaren-Faktors (HIF1) (Grafik 2). Dieser besteht aus 2 Untereinheiten a und b und vermittelt die Sauerstoffmessung in den Nieren. In Gegenwart von Sauerstoff werden die a-Ketten unter Beteiligung des VHL-Genproduktes abgebaut (3).
Die kongenitalen Erythrozytosen benötigen aus hämatologischer Sicht ebenfalls meistens keine spezifische Therapie und sind nicht mit erhöhtem Risiko für Thrombosen oder Transformation in eine andere Erkrankung verbunden. Die Ausnahme ist die Chuvah Erythrozytose (VHL-mutiert), da diese für eine Thrombosetendenz bekannt ist. Mit der Zunahme der Testung für die kongenitalen Erythrozytosen sowie einer besseren Datenlage, können hoffentlich zukünftig die klaren Managementstrategien etabliert werden. Um die Mutation, welche für die kongenitale Erythrozytose verantwortlich ist, zu untersuchen, muss eine molekulare Testung durchgeführt werden. Diese kann vom peripherenBlut erfolgen und mit NGS durchgeführt werden.