Acquired C1-esterase inhibitor deficiency and positive lupus anticoagulant accompanied by splenic marginal zone B-cell lymphoma
Sugisaki, K.; Itoh, K.; Tamaru, J.
Clinical and Experimental Rheumatology 25(4): 627-629
2007
ISSN/ISBN: 0392-856X PMID: 17888223 Document Number: 611158
A 70-year-old woman complained of mild shortness of breath. Laboratory findings revealed pancytopenia, positive lupus anticoagulant and severe hypocomplementemia without anti-nuclear or anti-DNA antibodies. After the failure of prednisolone treatment, an acquired C1-esterase inhibitor (C1-INH) deficiency was diagnosed. There were no episodes of angioedema or deep vein thrombosis. Three months later, extreme splenomegaly was detected. Lymph node biopsy suggested splenic marginal zone B-cell lymphoma. Acquired C1-INH deficiency due to a lymphoproliferative disorder should be considered as a possible diagnosis for patients with severe hypocomplementemia.