Acquired C1-esterase inhibitor deficiency and positive lupus anticoagulant accompanied by splenic marginal zone B-cell lymphoma.
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.
Sugisaki
K
K
Division of Internal Medicine, Kasukabe Central General Hospital, Saitama, Japan. kota_sugisaki@mbc.nifty.com
Itoh
K
K
Tamaru
J
J
D000368:Aged; D050718:Complement C1 Inhibitor Protein / Q000032:analysis*; D005260:Female; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000097:blood*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000097:blood*; D013160:Splenic Neoplasms / Q000097:blood
D050718:Complement C1 Inhibitor Protein; D016682:Lupus Coagulation Inhibitor