Acquired C1 inhibitor deficiency in a case of lymphosarcoma of the spleen. Reversal of complement abnormalities after splenectomy.
A patient with an extensive lymphosarcoma of the spleen without involvement of other lymphoid organs and hypogammaglobulinaemia showed the characteristic complement profile of an acquired C1-inhibitor deficiency. Both functional and immunochemical studies revealed extremely low levels of the inhibitor of C1-esterase. Correction of the low levels of early acting complement components and of the low C1-inhibitor level followed the splenectomy. In vitro tests showed that lymphosarcoma tissue pieces or cells were able to interact with complement, resulting in a depletion of the haemolytic activity. These findings provide evidence that tumour cells were responsible for the abnormalities of the complement system.
D000368:Aged; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003165:Complement System Proteins / Q000032:analysis; D005260:Female; D006801:Humans; D007122:Immunoelectrophoresis; D007075:Immunoglobulin M / Q000032:analysis; D008214:Lymphocytes / Q000648:ultrastructure; D008228:Lymphoma, Non-Hodgkin / Q000276:immunology* / Q000601:surgery / Q000648:ultrastructure; D008854:Microscopy, Electron; D011184:Postoperative Period; D013154:Spleen / Q000648:ultrastructure; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000276:immunology* / Q000601:surgery / Q000648:ultrastructure
D003174:Complement C1 Inactivator Proteins; D007075:Immunoglobulin M; D003165:Complement System Proteins