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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 Testosteronwert korreliert.
In der Gruppe mit Hormondefizit struggle die Mortalitätsrate quick doppelt so hoch wie in der
Gruppe mit normalen Testosteronspiegeln.
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.
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 Type 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 PV ist eine hämatologische, klonale Erkrankung, welche zu der Gruppe der myeloproliferativen Neoplasien (MPN) gehört.
Die PV-Patienten haben ein deutlich erhöhtes Risiko für venöse und arterielle thromboembolische (auch lebensbedrohliche) Ereignisse.
Zudem besteht bei ihnen ein Risiko für die Entwicklung einer Myelofibrose, einer Form der MPN mit einer ungünstigeren Prognose.
In seltenen Fällen kann die Erkrankung auch in eine akute myeloische Leukämie (AML) fortschreiten. Gemäss
der Klassifikation der Tumorerkrankungen der Weltgesundheitsorganisation (WHO) sowie
dem Internationalen Consensus der Klassifikation (ICC) der MPN aus dem Jahr
2022 gibt es klare diagnostische Kriterien für die PV (Tab. 1).

Prostatakrebszellen sind in der Lage, das Hormon Testosteron für ihr
eigenes Wachstum zu nutzen. Durch Hormonentzug kann es vorübergehend zu einem Stillstand des Tumorwachstums kommen.
Um eine im Vergleich flachere Testosteron-Wirkungskurve zu erreichen, stellten die Baseler Internisten auf das intramuskulär zu applizierende und länger wirksame Präparat
Testosteronundecanoat um. In Zukunft soll sich der Zeitpunkt
der Testosteron-Substitution am klinischen Allgemeinzustand
des Patienten sowie am peripheren Androgenspiegel orientieren. Zu beachten ist außerdem das erhöhte Prostatakarzinom-Risiko unter der Substitutionstherapie, dem Affected
Person wurde empfohlen, sich regelmäßig klinisch und sonografisch untersuchen lassen. Inzwischen lagen die
Ergebnisse der Knochenmarkspunktion vor, wonach ein normo- bis leicht hyperzelluläres Knochenmark mit
leichter Hyperplasie der Erythropoese bestand.
Es fanden sich kein morphologischer Anhalt für eine myeloproliferative Erkrankung und keine genetischen Aberrationen. “Die initial geschilderte abdominale Symptomatik interpretierten wir im Rahmen der Testosteronüberdosierung bei Regredienz im Verlauf als potenzielle Nebenwirkung”, so Kofler und Mitarbeiter.

Unser vorgeschlagener Diagnosealgorithmus für JAK2-unmutierte Polyzythämie bei Erwachsenen ist in Abbildung 1 dargestellt.

Zusammenfassend kann gesagt werden, dass die Polyzythämie und Erythrozytose wichtige Laborbefunde sind,
welche eine weitere Abklärung triggern sollten. Parameter, die
für die Kategorisierung dieser Artwork von Polyzythämie wegweisend
sind, umfassen den Epo-Spiegel im Serum und den P50-Wert.

Ursache der Testosteron-bedingten Polyglobulie ist vermutlich eine gehemmte Ausschüttung von Hepcidin,
das an der Regulation des Eisenstoffwechsels beteiligt ist.
Aus ungeklärten Gründen erhöht sich außerdem die Erythropoetin-Freisetzung.
Es gibt auch Berichte über eine vermehrte inflammatorische Aktivität unter Testosteron Bestellen Schweiz-Substitution – dies könnte eine Erklärung für
die beobachtete Leukozytose des Patienten sein. Zunächst jedoch behandelten die Schweizer Internisten den Patienten auf Grundlage der erhobenen Informationen wie einen Polycythaemia-Patienten. Er erhielt zur Thrombozytenaggregationshemmung Clopidogrel und wurde mehrfach zur Ader
gelassen.
Diese sekundären Polyzythämien sind eine physiologische Antwort auf die entstehende Gewebehypoxie.
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