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  • Title: Pathogenesis of hypofibrinogenemia in familial hemophagocytic lymphohistiocytosis.
    Author: Ooe K.
    Journal: Pediatr Pathol; 1991; 11(4):657-61. PubMed ID: 1946082.
    Abstract:
    In order to investigate the cause of hypofibrinogenemia in familial hemophagocytic lymphohistiocytosis (FHL), formalin-fixed and paraffin-embedded tissue sections of the spleen obtained at autopsy were examined by using immunohistochemical methods for the presence of fibrinogen antigens, and such antigens were detected in approximately 10% of the histiocytes in a diffuse staining pattern. This finding indicates uptake of fibrin and/or fibrinogen molecules by activated histiocytes and suggests that hypofibrinogenemia of FHL is caused by the direct action of activated histiocytes on factor X through Mac-1 receptors, subsequent activation of the common pathway of the coagulation protease cascade, and uptake of fibrin and/or fibrinogen molecules by such cells.
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