Hereditary Hemorrhagic Telangiectasia Showing Severe Anemia which was Successfully Treated with Estrogen.

Abstract

Hereditary hemorrhagic telangiectasia (Osier-Weber-Rendu disease) is a bleeding disorder attributed to a vascular developmental abnormality. It is transmitted as an autosomal dominant trait. A 63-year-old female was admitted because of repeated episodes of severe anemia which resulted from bleeding of telangiectases in the gastric mucosa. Conventional therapies including endoscopical microwavecoagulation and ethanol injection were not effective. The persistent anemia necessitated frequent blood transfusion. Estrogen was orally administrated and blood transfusion became unnecessary. Thus, estrogen therapy should be considered as one of the effective treatments for recurrent severe anemia due to hereditary hemorrhagic telangiectasia. (Internal Medicine 34: 589-592, 1995)

Telangiectasia hemorrágica hereditária: uma causa rara de anemia grave

“…(6,(19)(20) In the previously mentioned 9 studies, the most common cause of severe anemia in patients with HHT was found to be chronic gastrointestinal bleeding (in 70%), followed by epistaxis (in 25%). (9)(10)(11)(12)(13)(14)(15)(16)(17) Recurrent gastrointestinal tract hemorrhage occurs in up to one-third of individuals with HHT. The risk of such hemorrhage increases with advancing age, typically appearing in the fifth or sixth decade of life.…”

Vascular gastric anomalies as a cause of relapsing bleeding

“…Lečenje simptomatskog gastrointestinalnog krvarenja obuhvata različite endoskopske procedure (tople probe, argon-plazma koagulacija, laser). Hormonska terapija estrogenom i progesteronom, po nekim studijama, pokazala se uspešnom i može smanjiti potrebe za transfuzijom kod ovih bolesnika 10 . U toku su i ispitivanja koja govore u prilog pozitivnog efekta interferona-alfa na tok ove bolesti.…”

Die Argon-Plasma-Koagulation in der Behandlung von hereditären hämorrhagischen Teleangiektasien der Nasenschleimhaut

“…Zu den bisher versuchten Behandlungsstrategien zählen Hormontherapien mit Östrogenen und Progesteron, die Verabreichung von antifibrinolytischen Substanzen, die Embolisation und die Brachytherapie mit 192 Iridium [5,6,14,16,22]. Zu den bisher versuchten Behandlungsstrategien zählen Hormontherapien mit Östrogenen und Progesteron, die Verabreichung von antifibrinolytischen Substanzen, die Embolisation und die Brachytherapie mit 192 Iridium [5,6,14,16,22].…”