Therapeutic plasma exchange for the acute management of the catastrophic antiphospholipid syndrome: beta(2)-glycoprotein I antibodies as a marker of response to therapy.
We describe two patients with the catastrophic antiphospholipid syndrome associated with elevation of beta(2)-glycoprotein I antibodies and fulminant thrombotic diatheses. Both patients were treated with therapeutic plasma exchange (TPE), which resulted in a marked decrease in antibody titer accompanied by an improved clinical outcome in one patient (IgG antibody). In the second patient, the outcome was poor despite TPE (IgA antibody). There were no significant complications of TPE in either case. Because of the fulminant nature of the catastrophic antiphospholipid syndrome, we conclude that a trial of TPE is warranted for the acute management. Further studies are needed to clarify which patients may benefit from this treatment.
Flamholz
R
R
Department of Pathology-Blood Bank, The University of Chicago, Chicago, IL 60637, USA.
Tran
T
T
Grad
G I
GI
Mauer
A M
AM
Olopade
O I
OI
Ellman
M H
MH
McKinsey
J F
JF
Jeon
H R
HR
Baron
J M
JM
Baron
B W
BW
D000671:Amputation, Surgical; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000276:immunology / Q000628:therapy*; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology / Q000628:therapy*; D003131:Combined Modality Therapy; D003422:Critical Care / Q000379:methods*; D017809:Fatal Outcome; D005260:Female; D005385:Fingers / Q000098:blood supply; D005734:Gangrene; D006023:Glycoproteins / Q000276:immunology*; D006408:Hematoma, Subdural / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007511:Ischemia / Q000209:etiology; D007866:Leg / Q000098:blood supply / Q000473:pathology; D008875:Middle Aged; D010951:Plasma Exchange; D012008:Recurrence; D012594:Scleroderma, Localized / Q000150:complications; D013927:Thrombosis / Q000188:drug therapy / Q000209:etiology; D053482:beta 2-Glycoprotein I
D000925:Anticoagulants; D001323:Autoantibodies; D006023:Glycoproteins; D007166:Immunosuppressive Agents; D053482:beta 2-Glycoprotein I