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Generalized Proteolysis in a Young Woman with Weber‐Christian Disease (Nodular Nonsuppurative Panniculitis)
Author(s) -
Henriksson P.,
Hedner U.,
Nilsson I. M.,
Nilsson P.G.
Publication year - 1975
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1975.tb02707.x
Subject(s) - fibrin , fibrinogen , plasmin , proteolytic enzymes , proteolysis , thrombin , clotting factor , factor xiii , medicine , pathology , immunology , platelet , enzyme , chemistry , biochemistry
A patient with Weber‐Christian disease (syn. nodular nonsuppurative panniculitis) is reported. The generalized cellular destruction in this patient resulted in liberation of proteolytic enzymes into the circulation, which led to multiple haemostatic disturbances with haemorrhagic diathesis. The most prominent haemostatic defects were thrombocytopenia with a normal life span of isologous platelets, high levels of AHF‐related antigen, hypofibrinogenaemia with short fibrinogen survival, low levels of Factor XIII (fibrin stabilizing factor = FSF) and increased amounts of fibrin/fibrinogen degradation products (FDP). Proteolytic enzymes, other than thrombin and plasmin which especially degrade Factor XIII and fibrinogen, derived from destroyed cells (probably leukocytes) seem to have been involved in the pathogenesis of the bleeding disorder in this patient.