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Die klinische bedeutung von protein-C- und -S-Mangelzuständen für das chirurgische patientenkollektiv

The clinical impact of protein C and S deficiency for the surgical patient population

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Summary

Protein C and S are important factors in blood coagulation reported in many papers about people who suffered from thrombembolic diseases related to inherited or acquired deficiencies. Homozygous protein C/S deficiency is lethal in most cases without therapy. Heterozygous deficiency is moderate and complications occur between the 20.–50. year of age. Acquired protein C/S deficiency is a strong parameter for liver function. The typical clinical manifestations of protein C/S deficiencies are superficial and deep leg vein thrombosis, thrombosis of the mesenterial, cerebral, renal and axillary veins, portal vein thrombosis and pulmonary embolism. Most of the affected people live disease free over a longer period and develop thrombembolic complications during and after trauma, surgical interventions, pregnancy and puerperium. We report our experience with a 60 years old male who had developed a severe bilateral iliofemoral vein thrombosis with signs of pulmonary embolism after total hip replacement. An extented functional protein C deficiency (type II) was investigated by coagulation tests (Protein C Reagent, coagulometric from Behring Institute). A second female patient developed a descending iliofemoral vein thrombosis during pregnancy. Venous thrombectomy with arteriovenous fistula was performed, but reocclusion occurred after delivery. Redo-surgery was undertaken and a second reocclusion took place 10 days later. Further lysis therapy was not able to reopen the venous system. Whereas immunological and functional protein C levels showed normal ranges, the functional protein S level was markedly reduced (IL-Instrumentation Laboratory Protein S-Test).

Zusammenfassung

Die zentrale Stellung des Protein C und S innerhalb des Blutgerinnungssystems ist durch eine Vielzahl von Patienten belegt, bei denen ein angeborener oder erworbener Mangel zu gesteigerten thrombembolischen Komplikationen geführt hat. Wahrend der angeborene, homozygote Protein-C- und -S-Mangel ohne Therapie kaum mit dem Leben vereinbar ist, manifestiert sich der heterozygote Defekt als abgeschwächte Form meist spontan zwischen dem 20. and 50. Lebensjahr. Erworbener Protein-C-Mangel ist ein starker Indikator für gestörte Leberfunktion und tritt unter bestimmten Bedingungen auf. Typisch für Protein-C- and -S-Mangelzustände sind venöse Thrombosen der oberflächlichen und tiefen Beinvenen, der Mesenterialvenen, der Zerebralvenen, der Nieren- und Axillarvenen Bowie Pfortaderthrombosen und Pulmonalembolien. Viele der Merkmalträger leben über längere Zeit völlig asymptomatisch und entwickeln im Anschluß an Traumen, Operationen, Schwangerschaft und Wochenbett ausgedehnte thrombembolische Komplikationen. Wir berichten über einen 60jährigen, männlichen Patienten, der nach totalem Hüftgelenkersatz zunächst links, darn rechts eine fulminante, rezidivierende Thrombose der Bein-Becken-Venen mit den klinischen Zeichen der Pulmonalembolie bot. Die Gerinnungsfaktorenanalyse ergab einen ausgeprägten, funktionellen (Typ II) Protein-C-Mangel (Protein-C-Reagenz koagulometrisch, Behring Institut). Eine 2. Patientin entwickelte eine deszendierende Beckenvenenthrombose gegen Ende der Schwangerschaft. Es erfolgte eine venöse Thrombektomie mit temporärer AV-Fistel, die sich post partum wiederum verschloß. Eine nochmalige venöse Thrombektomie mit neuerlichem Reverschluß nach der Entlassung komplizierte den Verlauf. Eine darauffolgende Lysetherapie konnte die Wiedereröffnung der Venenstrombahn nicht mehr erzielen. Wahrend sich ein normaler Protein-C-Gehalt nachweisen ließ, war der funktionelle (Typ II) Protein- S-Gehalt (IL-Instrumentation Laboratory Protein S-Test) erniedrigt.

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  1. Universitätsklinik für Chirurgie, Auenbruggerplatz 29, A-8036, Graz, Osterreich

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Rabl, H., Fruhwirth, H. Die klinische bedeutung von protein-C- und -S-Mangelzuständen für das chirurgische patientenkollektiv. Langenbecks Arch Chir 377, 75–80 (1992). https://doi.org/10.1007/BF00184339

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