title: Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies.
abstract: OBJECTIVE
  To analyse the clinical and laboratory features of patients with thrombotic microangiopathic haemolytic anaemia (TMHA) associated with antiphospholipid antibodies (aPL).
  
  
  METHODS
  A computer assisted (PubMed) search of the literature was performed to identify all cases of TMHA associated with aPL from 1983 to December 2002.
  
  
  RESULTS
  46 patients (36 female) with a mean (SD) age at presentation of TMHA of 34 (15) years were reviewed. Twenty eight (61%) patients had primary antiphospholipid syndrome (APS). TMHA was the first clinical manifestation of APS in 26 (57%) patients. The clinical presentations were haemolytic-uraemic syndrome (26%), catastrophic APS (23%), acute renal failure (15%), malignant hypertension (13%), thrombotic thrombocytopenic purpura (13%), and HELLP (haemolysis, elevated liver enzymes, and low platelet count in association with eclampsia) syndrome (4%). Lupus anticoagulant was detected in 86% of the episodes of TMHA, and positive anticardiolipin antibodies titres in 89%. Steroids were the most common treatment (69% of episodes), followed by plasma exchange (PE) (62%), anticoagulant or antithrombotic agents (48%), immunosuppressive agents (29%), and immunoglobulins (12%). Recovery occurred in only 10/29 (34%) episodes treated with steroids, and in 19/27 (70%) episodes treated with PE. Death occurred in 10/46 (22%) patients.
  
  
  CONCLUSIONS
  The results emphasise the need for systematic screening for aPL in all patients with clinical and laboratory features of TMHA. The existence of TMHA in association with an APS forces one to rule out the presence of the catastrophic variant of this syndrome. PE is indicated as a first line of treatment for all patients with TMHA associated with aPL.
authors.affiliation: Department of Autoimmune Diseases, Institut Clínic d'Infeccions i Immunologia, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Catalonia, Spain.
authors.forename: G
authors.initials: G
authors.lastname: Espinosa
authors.forename: S
authors.initials: S
authors.lastname: Bucciarelli
authors.forename: R
authors.initials: R
authors.lastname: Cervera
authors.forename: M
authors.initials: M
authors.lastname: Lozano
authors.forename: J-C
authors.initials: JC
authors.lastname: Reverter
authors.forename: G
authors.initials: G
authors.lastname: de la Red
authors.forename: V
authors.initials: V
authors.lastname: Gil
authors.forename: M
authors.initials: M
authors.lastname: Ingelmo
authors.forename: J
authors.initials: J
authors.lastname: Font
authors.forename: R A
authors.initials: RA
authors.lastname: Asherson
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D017152:Antibodies, Antiphospholipid / Q000032:analysis*; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000276:immunology / Q000628:therapy; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology / Q000628:therapy; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants; D005343:Fibrinolytic Agents; D007136:Immunoglobulins; D007166:Immunosuppressive Agents; D013256:Steroids