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Eltrombopag in systemic lupus erythematosus with antiphospholipid syndrome: thrombotic events

Sir, Maroun et al. suggested that eltrombopag is useful to treat immune thrombocytopenic purpura (ITP) in systemic lupus erythematosus (SLE) in order to spare steroids, but they conclude that little data is available in patients with antiphospholipid syndrome (APS). We would like to focus on the case of a patient with APS who had severe thrombotic events after starting eltrombopag. This patient was 61 years old and had been diagnosed as having APS because of three fetal losses, two pulmonary embolisms and five deep venous thromboses. Antivitamin K treatment was given and no thrombosis occurred for more than 10 years. Lupus anticoagulant was positive and persistent in three samples (in 2000, 2002 and 2006), but there was no anticardiolipin or anti-b2gp1 antibody. ANA were mildly positive (1/250) and no anti-DNA or anti-ENA antibody was found. Anti-gpIIbIIIa antibodies were present. She was treated over 10 years for ITP with steroids, cyclophosphamide, azathioprine, intravenous immunoglobulins and splenectomy. However, she was hospitalized for diffuse petechial eruption with very low platelet count (4000/mm) in January 2011. Fibrinogen rate was 4 g/l and factor V 100%, so disseminated intravascular coagulation was unlikely. Treatment was started with eltrombopag (50mg/d) together with intravenous immunoglobulins. One month later, platelet count was 266,000/mm. On 27 February 2011, she was hospitalized for dyspnoea. The international normalized ratio (INR) was 4.97 and platelet count was 119,000/mm. Computed tomography (CT) scan showed pulmonary embolism of the proximal left pulmonary artery and lobar and segmental branches. The left adrenal gland was suspected of haemorrhagic necrosis. The day after, she experienced coma and needed nasotracheal intubation. Cerebral CT scan found frontotemporal hematoma, and unfortunately the patient died with brain herniation. This patient had had no thrombosis for more than 10 years, and presented with proximal pulmonary embolism 1 month after starting eltrombopag despite low platelet count and INR above the target. The mechanisms involved are speculative, but APS has been shown to be associated with platelet activation; Sperati suggested that reconstitution of circulating platelets leads to a worsening of thrombosis in a setting of platelet activation. Furthermore, other events might also be related to thrombosis: adrenal necrosis and cerebral haematoma, which might be an ischaemic vascular accident complicated by haemorrhage. We suggest that eltrombopag should be used with caution in patients with APS.