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 respo...

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Veröffentlicht in:Clinical rheumatology 2007-10, Vol.26 (10), p.1737-1740
Hauptverfasser: Lazurova, I, Macejova, Z, Tomkova, Z, Remenar, F, Boor, A, Lazur, J, Roland, R, Rovensky, J, Asherson, R A
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container_end_page 1740
container_issue 10
container_start_page 1737
container_title Clinical rheumatology
container_volume 26
creator Lazurova, I
Macejova, Z
Tomkova, Z
Remenar, F
Boor, A
Lazur, J
Roland, R
Rovensky, J
Asherson, R A
description 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.
doi_str_mv 10.1007/s10067-006-0487-8
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subjects Anemia
Antiphospholipid Syndrome - diagnosis
Antiphospholipid Syndrome - mortality
Antiphospholipid Syndrome - pathology
Cerebral Hemorrhage
Diagnosis, Differential
Epoprostenol - pharmacology
Extremities - pathology
Fatal Outcome
Gastroenteritis - complications
Humans
Male
Middle Aged
Necrosis - pathology
Oxygen
Oxygen - metabolism
Thrombosis - diagnosis
Thrombosis - mortality
Thrombosis - pathology
Vascular Diseases - diagnosis
Vascular Diseases - mortality
Vascular Diseases - pathology
title Severe limb necrosis: primary thrombotic microangiopathy or "seronegative" catastrophic antiphospholipid syndrome? A diagnostic dilemma
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