ACQUIRED C1 INHIBITOR DEFICIENCY IN A CASE OF LYMPHOSARCOMA OF THE SPLEEN - REVERSAL OF COMPLEMENT ABNORMALITIES AFTER SPLENECTOMY

  • 1 January 1979
    • journal article
    • research article
    • Vol. 37  (3) , 523-531
Abstract
A patient with an extensive lymphosarcoma of the spleen without involvement of other lymphoid organs and hypogammaglobulinemia showed the characteristic complement [C] profile of an acquired C.hivin.1-inhibitor deficiency. Functional and immunochemical studies revealed extremely low levels of the inhibitor of C.hivin.1-esterase. Correction of the low levels of early acting C components and of the low C.hivin.1-inhibitor level followed the splenectomy. Lymphosarcoma tissue pieces or cells could interact with C in vitro, resulting in a depletion of the hemolytic activity. Tumor cells were apparently responsible for the abnormalities of the C system.