Glossary entry

German term or phrase:

Hemmkörper-Hämophilie

English translation:

Hemophilia with inhibitors

Added to glossary by Christine Lam
Jan 13, 2007 22:39
17 yrs ago
4 viewers *
German term

Hemmkörper-Hämophilie

German to English Medical Medical (general)
Diagnosen eines Herzpatienten
Koronare Dreigefäßerkrankung
Intermittierendes Vorhofflimmern
Hemmkörper-Hämophilie
Karotisstenosen bds.
Penicillinallergie
usw.

Discussion

Christine Lam (asker) Jan 14, 2007:
I have one more sentence in which the term occurs: Bei derzeit gut therapierter Hemmkörperhämophilie mit Nachweis einer 100%igen Faktor VIII-Aktivität konnte die vorgesehene Koro. durchgeführt werden.
Christine Lam (asker) Jan 14, 2007:
thank you all for your help. Marlou, I am not completely convinced that this should be left as is, but let´s wait and hopefully hear from some more experts in the field.
Anton Baer Jan 13, 2007:
This may help. (FVIII antibodies.)
http://www.igh.info/content/view/258/35/
Die Bildung von FVIII-Antikörpern (Hemmkörper) bei manchen Patienten ist noch immer ein ungelöstes Problem in der Hämophilie-Therapie...

Proposed translations

+2
5 hrs
Selected

Hemophilia with inhibitors

I'll risk an answer and await the wakening of the experts in Europe on Sunday morning.

http://www.novoseven-us.com/patient_hemophilia/inhibitors_Wh...

"When someone is factor VIII - or factor IX - deficient, clotting factor treatment must be brought into the blood through injection or infusion to make up for the factor VIII or factor IX that they are missing. In some cases, their body may not recognize the treatment as a helpful substance, and their immune system will develop antibodies—inhibitors—to it. When these antibodies are present, they attack and neutralize the factor VIII or IX that has been injected or infused. This causes the bleeding to continue. "

http://www.springerlink.com/content/hn82w2tg1q027120/

Zusammenfassung: Eine erworbene Hämophilie stellt eine seltene Komplikation bei autoimmunen Erkrankungen und Malignomen dar... Wir berichten über eine Patientin mit akuter intrakranieller Blutung aufgrund einer erworbenen **Hemmkörperhämophilie** Faktor VIII betreffend.

Summary: Acquired hemophilia is a rare complication in autoimmune disorders and malignancies. .. We report a patient with acute intracerebral hemorrhage due to **acquired hemophilia with factor VIII inhibition**.

http://www.springerlink.com/content/fdyk8176y2u08gud/

Wir berichten über einen 47-jährigen Patienten mit seit Jahren bestehender arterieller Hypertonie und dialysepflichtiger, terminaler Niereninsuffizienz.... Die erhobenen Befunde ergaben eine Faktor-VIII-Hemmkörperhämophilie, in den Immunfluoreszenztests zeigte sich außerdem ein dezentes Pemphigoid-typisches IgG-Muster.

A 47-year old patient who had been suffering from hypertension and chronic renal failure for many years developed progressive extensive haemorrhagic erosions of the mouth within 3 months and less severe erosions of the genital and nasal mucosa... Laboratory investigations demonstrated circulating antibodies against factor VIII while direct and indirect immunofluorescent microscopy showed discrete tissue-bound and circulating IgG reactive with the epidermal basement membrane in a pemphigoid-like fashion...

Finally, from http://de.wikipedia.org/wiki/Inhibitor

"Inhibitoren (Hemmkörper) sind eine gefürchtete Komplikation der Hämophilie (Bluterkrankheit). Sie verhindern die Wirksamkeit des Faktor VIII, ein Eiweiß, das unverzichtbar für die Blutgerinnung ist. Die Elimination der Hemmkörper ist durch eine langandauernde Hochdosierung von Faktor VIII möglich."

So some cases of Hemophilia would be called "Hemophilia due to factor VIII inhibition/due to factor VIII", and others "Hemophilia due to factor IX inhibition/due to factor IX'...

But essentially it's "hemophilia with inhibitors", if those specific factors aren't noted.
Peer comment(s):

agree Anne Schulz : ...now that I'm awake...
3 hrs
agree MMUlr : I prefer this one, too, see http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid... (the NHF uses at the end of the text "hemophilia with inhibitors" (also NovoNordisk does), it may be congenital or acquired.
4 hrs
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4 KudoZ points awarded for this answer. Comment: "thank you for risking it!"
23 mins

Hemmkorper-Heamophilie

I suggest that there is no English term, it is a German name for a disease.
Maybe you can check it out in the link en judge voor yourself.

The same year, Deutsch’4 published a monograph on the disease,
including one case of his own. He suggested the name “Hemmkorper-
Haemophihie”.


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Note added at 42 mins (2007-01-13 23:21:55 GMT)
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Hemmkorper-Hemophilia is a Hemophilia-like disease as I understand fron the article.
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+1
1 hr

Acquired hemophelia

Here's what I found:
Dealing with acquired hemophilia -- Kitchens 103 (12): 4375 -- BloodAs such, acquired factor VIII inhibitors giving rise to acquired hemophilia may be added to the list of other autoimmune hematologic disorders for which ...
www.bloodjournal.org/cgi/content/full/103/12/4375 - Similar pages

Peer comment(s):

agree milinad
1 day 3 hrs
Thank you
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