+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Severe limb necrosis: primary thrombotic microangiopathy or "seronegative" catastrophic antiphospholipid syndrome? A diagnostic dilemma

Severe limb necrosis: primary thrombotic microangiopathy or "seronegative" catastrophic antiphospholipid syndrome? A diagnostic dilemma

Clinical Rheumatology 26(10): 1737-1740

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.

Please choose payment method:

(PDF emailed within 0-6 h: $19.90)

Accession: 050293867

Download citation: RISBibTeXText

PMID: 17256103

DOI: 10.1007/s10067-006-0487-8

Related references

Catastrophic antiphospholipid syndrome with severe acute thrombotic microangiopathy and hemorrhagic complications. Case Reports in Medicine 2013: 915309, 2013

Catastrophic antiphospholipid antibody syndrome in a child with thrombotic microangiopathy. Indian Journal of Nephrology 22(4): 310-313, 2012

Thrombotic microangiopathy with liver, gut, and bone infarction (catastrophic antiphospholipid syndrome) associated with HELLP syndrome. Clinical Rheumatology 24(2): 166-168, 2005

Catastrophic antiphospholipid syndrome presenting with renal thrombotic microangiopathy and diffuse proliferative glomerulonephritis. Clinical and Experimental Rheumatology 24(1): 110, 2006

Catastrophic Antiphospholipid Syndrome Presenting as Congestive Heart Failure in a Patient with Thrombotic Microangiopathy. Texas Heart Institute Journal 46(1): 48-52, 2019

Diagnostic dilemma: Severe thrombotic microangiopathy in pregnancy. Journal of the Intensive Care Society 18(4): 348-351, 2017

The primary, secondary, catastrophic, and seronegative variants of the antiphospholipid syndrome: a personal history long in the making. Seminars in Thrombosis and Hemostasis 34(3): 227-235, 2008

Antiphospholipid syndrome without antiphospholipid antibodies at the time of the thrombotic event: transient 'seronegative' antiphospholipid syndrome?. Clinical and Experimental Rheumatology 15(5): 541-544, 1997

Primary antiphospholipid syndrome paps associated with renal thrombotic microangiopathy tma report of 4 cases. Journal of the American Society of Nephrology 2(3): 267, 1991

A case of primary antiphospholipid antibody syndrome with acute renal failure showing thrombotic microangiopathy. American Journal of Nephrology 19(5): 594-598, 1999

Primary antiphospholipid syndrome presenting as thrombotic microangiopathy: Successful treatment with steroids, plasma exchange and anticoagulants. Indian Journal of Nephrology 21(4): 280-282, 2011

Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet. Haematology 3(5): E237-E245, 2016

A case of seronegative catastrophic antiphospholipid antibody syndrome. Connecticut Medicine 77(2): 81-83, 2013

Livedo racemosa and digital necrosis in a patient with primary seronegative antiphospholipid syndrome and fibromuscular dysplasia of peripheral arteries. British Journal of Dermatology 157(2): 389-392, 2007

Thrombotic microangiopathy and the antiphospholipid syndrome. Lupus 19(13): 1569-1572, 2010