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Generalisierte plasmatische Hypercoagulabilität und pulmonale hyaline Membranen beim Erwachsenen

Generalised plasmatic hypercoagulability and pulmonary hyaline membranes in the adult

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Summary

1. The pulmonary hyaline membranes of the adult are much more common than admitted so far. In 42 of 833 autopsies (5%) performed after the 7th day of life (end of the perinatal period), more or less extensive hyaline membranes have been observed in one or more lobes.

2. Disseminated intravascular microthrombi—a morphological equivalent of a generalised hypercoagulability—were found in 71 of 93 autopsies. In most of these, no relation between the basic disease and the frequency of hyaline membranes could be established. However, in 72 of 93 cases, the formation of hyaline membranes was preceded by a prolonged shock of traumatic, postoperative, cardiogenic, allergic-hyperergic, septic, endotoxic or infectious-toxic origin or by an operation with extracorporal circulation. The remaining cases showed malignant neoplasms (carcinoma of the pancreas, colon, stomach, and prostate gland, malignant teratoid, multiform glioblastoma, promyelocytic leukemia, liver cirrhosis, congenital cyanotic heart diseases and in one case a premature separation of the placenta.

3. It is assumed that a generalised activation of coagulation with formation of circulating fibrin monomeres is the common pathogenic principle to all cases with pulmonary hyaline membranes. The extravasation of the plasmatic fibrin monomeres into the alveoli and into the terminal bronchioles and their extravascular polymerisation lead to the formation of the pulmonary hyaline membranes. The frequency of hyaline membranes in the adult is due to the frequency of a generalised plasmatic hypercoagulability which is associated with cases of shock (of different etiology), malignant tumors, promyelocytic leukemias, congenital cyanotic heart disease, liver cirrhosis and subpartual bleeding complications at birth.

4. Alike pulmonary atelectases, pulmonary hyaline membranes are the morphological equivalent of a syndrome of respiratory distress in the adult, an acute respiratory insufficiency due to circulatory shock. The relationships between shock, hyaline membranes, inhibition and/or disturbance of the synthesis of the surfactant and respiratory insufficiency are discussed.

Zusammenfassung

1. Pulmonale hyaline Membranen des Erwachsenen sind häufiger, als bislang vermutet wurde. 42 von 833 Obduktionsfällen (5%) zeigten jenseits des 7. postnatalen Lebenstages (Ende der Perinatalperiode) mehr oder weniger ausgedehnte hyaline Membranen in einem oder in mehreren Lungenlappen.

2. Bei 71 von 93 Obduktionsfällen mit pulmonalen hyalinen Membranen ließen sich disseminierte intravasale Mikrothromben als morphologische Äquivalente einer generalisierten plasmatischen Hypercoagulabilität nachweisen. Beziehungen zwischen der Grundkrankheit der 93 Obduktionsfälle und der Häufigkeit pulmonaler hyaliner Membranen ließen sich nicht erkennen. Bei 72 der 93 Obduktionsfälle ging der Ausbildung der pulmonalen hyalinen Membranen jedoch ein protrahierter (traumatischer, postoperativer, kardiogener, allergisch-hyperergischer, septischer bzw. bakteriell-endotoxischer und infektiös-toxischer) Schock oder eine Operation im extracorporalen Kreislauf voraus, in den übrigen Fällen fanden sich maligne Tumoren (Pankreas-, Magen-, Colon- und Prostatacarcinome, malignes Teratoid, Glioblastoma multiforme), Promyelocytenleukämien, Lebercirrhosen, cyanotischen Herzfehlern sowie in einem Fall eine vorzeitige Lösung der Placenta.

3. Als gemeinsames pathogenetisches Prinzip aller Obduktionsfälle mit pulmonalen hyalinen Membranen muß eine generalisierte Aktivierung der Gerinnung mit Ausbildung intravasal zirkulierender Fibrinmonomere gelten. Die Extravasation der plasmatischen Fibrinmonomere im Bereich der Alveolen und Bronchioli terminales und ihre extravasale Polymerisation führt zur Ausbildung der pulmonalen hyalinen Membranen. Die Häufigkeit der hyalinen Membranen des Erwachsenen resultiert aus der Häufigkeit einer generalisierten plasmatischen Hypercoagulabilität bei Schockfällen verschiedenster Ätiologie, bei malignen Tumoren, Promyclocytenleukämien, cyanotischen Herzfehlern, Lebercirrhosen und subpartualen Geburtskomplikationen.

4. Pulmonale hyaline Membranen sind wie pulmonale Atelektasen morphologisches Äquivalent eines im Gefolge eines Kreislaufschocks auftretenden Atemnotsyndroms der Erwachsenen, einer akuten respiratorischen Insuffizienz. Die Wechselbeziehungen zwischen Schock, hyalinen Membranen, Hemmung und Synthesestörung des Anti-Atelektase-Faktors und respiratorischer Insuffizienz werden diskutiert.

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  1. Institut für Allgemeine Pathologie und pathologische Anatomie der Universität Heidelberg, Germany

    U. Bleyl, K. Heilmann & D. Adler

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  1. U. Bleyl
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  2. K. Heilmann
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  3. D. Adler
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Mit Unterstützung durch die Deutsche Forschungsgemeinschaft.

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Bleyl, U., Heilmann, K. & Adler, D. Generalisierte plasmatische Hypercoagulabilität und pulmonale hyaline Membranen beim Erwachsenen. Klin Wochenschr 49, 71–81 (1971). https://doi.org/10.1007/BF01497303

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  • DOI: https://doi.org/10.1007/BF01497303

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