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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 
 
 
 
 

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.[1]

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