Severe limb necrosis: primary thrombotic microangiopathy or "seronegative" catastrophic antiphospholipid syndrome? A diagnostic dilemma.
An episode of gastroenteritis triggered severe necrosis of all extremities in a previously asymptomatic male. Hepatic and renal involvement were also manifest, while the hematological picture was one of thrombotic microangiopathic hemolytic anemia. Antiphospholipid antibodies were negative. He responded well to a combination of plasma exchange, anticoagulation (heparin), parenteral steroids, and antibiotics, as well as vasodilators (prostacycline) and hyperbaric oxygen, but died because of a cerebral hemorrhage. The differential diagnosis included thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome, or seronegative catastrophic antiphospholipid (Asherson's) syndrome. The dangers of administering such a combination of therapies with anticoagulation, as well as vasodilatation (prostacycline) and hyperbaric oxygen, are highlighted by the case report and emphasized.
Lazurova
I
I
1st Department of Internal Medicine, Medical Faculty, University Kosice, tr. SNP 1, 040 11, Kosice, Slovakia. lazurova@central.medic.upjs.sk
Macejova
Z
Z
Tomkova
Z
Z
Remenar
F
F
Boor
A
A
Lazur
J
J
Roland
R
R
Rovensky
J
J
Asherson
R A
RA
D000740:Anemia; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D002543:Cerebral Hemorrhage; D003937:Diagnosis, Differential; D011464:Epoprostenol / Q000494:pharmacology; D005121:Extremities / Q000473:pathology*; D017809:Fatal Outcome; D005759:Gastroenteritis / Q000150:complications; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis / Q000473:pathology*; D010100:Oxygen / Q000378:metabolism; D013927:Thrombosis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D014652:Vascular Diseases / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology
D011464:Epoprostenol; D010100:Oxygen