Catastrophic Antiphospholipid Syndrome - Plasmapheresis

1/06/03 (Arellano)

Question: Is plasmapheresis an effective therapy for the catastrophic antiphospholipid syndrome?

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 <1>

Unique Identifier:11882732

Authors: Levine JS. Branch DW. Rauch J.

Institution: Department of Medicine, Section of Nephrology, University of Chicago, USA.

Title: The antiphospholipid syndrome.[comment]. [Review] [96 refs]

Source: New England Journal of Medicine. 346(10):752-63, 2002 Mar 7.

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Unique Identifier:10961585

Authors: Asherson RA. Cervera R.

Institution: Department of Medicine, The Groote School Hospital, University of Cape Town School of Medicine, South Africa. ashspot@icon.co.za

Title: Catastrophic antiphospholipid syndrome. [Review] [13 refs]

Source: Current Opinion in Hematology. 7(5):325-9, 2000 Sep.

Abstract: In its classic presentation, the antiphospholipid syndrome manifests a combination of venous or arterial thrombosis and fetal loss, accompanied by elevations of antibodies directed toward negatively charged phospholipids, as measured by anticardiolipin antibody assays and/or positive lupus anticoagulant tests. The manifestations often include a moderate thrombocytopenia and, less commonly, hemolysis. In contrast, a less frequently encountered subset of the antiphospholipid syndrome, termed the "catastrophic" antiphospholipid syndrome, affects mainly small vessels predominantly supplying organs. The thrombocytopenia is usually marked, and a Coombs positive microangiopathic-type anemia may accompany the condition. Features of disseminated intravascular coagulation may be evident in some patients. It is fatal in approximately 50% of cases reported. Treatment should include not only adequate anticoagulation with intravenous heparin but also full doses of intravenous corticosteroids, to offset the systemic inflammatory response syndrome that occurs as a result of the extensive tissue damage, and plasmapheresis, using fresh frozen plasma. Parenteral antibiotics should be administered early if infection is suspected. [References: 13]

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Unique Identifier:9653431

Authors: Asherson RA. Cervera R. Piette JC. Font J. Lie JT. Burcoglu A. Lim K. Munoz-Rodriguez FJ. Levy RA. Boue F. Rossert J. Ingelmo M.

Institution: Rheumatic Diseases Unit, University of Cape Town School of Medicine, South Africa.

Title: Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. [Review] [59 refs]

Source: Medicine. 77(3):195-207, 1998 May.

Abstract: We analyzed the clinical and laboratory characteristics of 50 patients with catastrophic antiphospholipid syndrome (APS) (5 from our clinics and 45 from a MEDLINE computer-assisted review of the literature from 1992 through 1996). Thirty-three (66%) patients were female and 17 (34%) were male. Twenty-eight (56%) patients had primary APS, 15 (30%) had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 1 (2%) had rheumatoid arthritis. Mean age of patients in this series was 38 +/- 14 years (range, 11-74 yr). Three (6%) patients developed the clinical picture of catastrophic APS under the age of 15 years, and 11 (22%) were 50 years old or more. In 11 (22%) patients, precipitating factors contributed to the development of catastrophic APS (infections in 3, drugs in 3, minor surgical procedures in 3, anticoagulation withdrawal in 2, and hysterectomy in 1). The presentation of the acute multi-organ failure was usually complex, involving multiple organs simultaneously or in a very short period of time. The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Thrombocytopenia was reported in 34 (68%) patients, hemolytic anemia in 13 (26%), disseminated intravascular coagulation in 14 (28%), and schistocytes in 7 (14%). The following antibodies were detected: lupus anticoagulant (94%), anticardiolipin antibodies (94%), anti-dsDNA (87% of patients with SLE), antinuclear antibodies (58%), anti-Ro/SS-A (8%), anti-RNP (8%), and anti-La/SS-B (2%). Anticoagulation was used in 70% of the patients, steroids in 70%, plasmapheresis in 40%, cyclophosphamide in 34%, intravenous gammaglobulins in 16%, and splenectomy in 4%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 25 of the 50 (50%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Among the 20 patients who received the combination of anticoagulation, steroids, and plasmapheresis or intravenous gammaglobulins, recovery occurred in 14 (70%) patients. The use of ancrod and defibrotide appeared to be effective in the 2 respective patients in whom they were used. [References: 59] CAS Registry/EC Number 0 (Antibodies, Antinuclear). 0 (Immunoglobulin G). 0 (Immunoglobulin M).