Mesenteric vein thrombosis associated with a familial deficiency of free protein S.
Signs and symptoms of an acute intra-abdominal emergency developed in a 26-year-old man; this emergency prompted surgical exploration. It was discovered that he had a devitalized small bowel as a result of mesenteric vein thrombosis. An extensive evaluation for hypercoagulable states disclosed that he had a normal total protein S concentration, but by assay and on crossed immunoelectrophoresis studies, he had a decreased concentration of free protein S. A family study demonstrated protein S deficiency in his father and in a sister. On a subsequent determination, the propositus had a decreased concentration of total protein S. This case illustrates the association of protein S deficiency with mesenteric thrombosis and serves to caution that a single determination of total protein S is not an adequate evaluation when the index of suspicion for a hypercoagulable state is high.
Clark
D A
DA
Department of Medicine, Veterans Affairs Medical Center, Albuquerque, NM.
Williams
W L
WL
Marlar
R A
RA
D000328:Adult; D001798:Blood Proteins; D006023:Glycoproteins / Q000172:deficiency*; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D007583:Jejunum / Q000098:blood supply / Q000473:pathology; D008297:Male; D008642:Mesenteric Veins; D017293:Protein S; D013927:Thrombosis / Q000209:etiology* / Q000235:genetics / Q000473:pathology
D001798:Blood Proteins; D006023:Glycoproteins; D017293:Protein S