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.

Renal Thrombotic Microangiopathies Induced by Severe Hypertension

“…In one series of 56 patients with malignant hypertension, microangiopathic hemolytic anemia was present in 23 patients (8). Espinosa et al (9) reported that 13% of patients with systemic TMA showed malignant hypertension. On the other hand, the frequency of intrarenal TMA with malignant hypertension has not been well described (10,11).…”

Update on hemolytic uremic syndrome: Diagnostic and therapeutic recommendations

“…Several reports have noted the relationship between TMA and the presence of antiphospholipid antibodies [151] . HUS and, less commonly, TTP and HELLP syndrome, may be the presenting manifestation [152] . The existence of TMA in association with APS forces to rule out the catastrophic variant of the syndrome.…”

Catastrophic antiphospholipid syndrome (CAPS)

“…However, the efficacy of PE has been shown in other thrombotic microangiopathy and since CAPS is a critical situation with a high rate of mortality, it is recommended as part of the CAPS treatment [15].…”