Roussotte M, Gerfaud-Valentin M, Hot A, et al. - A high rate of haematological abnormalities was evident in patients with immune thrombocytopenia with clinical significance (ITPCS) associated with systemic lupus erythematosus (SLE), and a higher morbidity was seen in major bleeding event (MBG) cases. High heterogeneity in thrombocytopenia management was observed and many options seem viable.

• In this retrospective multicentre study, SLE patients with ≥1 ITPCS were analyzed.

• Of 90 patients included, 25 had received a diagnosis of ITP prior to SLE; they had high rate of autoimmune haemolytic anaemia (15%), antiphospholipid antibody (62%), and antiphospholipid syndrome (19%).

• MBG occurred in 28%; this group exhibited significantly more bleeding at ITP diagnosis and higher bleeding scores, and serositis and thrombosis during follow-up, and needed significantly more treatment lines, transfusions, and hospitalizations.

• A significantly more restricted SLE phenotype (cutaneous and/or articular) was obvious in 12% of patients who had no bleeding event.

• In one third of patients, ITPCS overall response resulted from corticosteroids and hydroxychloroquine.

• With rituximab (n = 34), azathioprine (n = 19), mycophenolate mofetil (n = 8), thrombopoietin-receptor agonists (n = 16), and splenectomy (n = 19), the achieved median relapse-free survival was 53, 31.5, 61, 24.5, and 78 months, respectively.

• In 4 patients, thrombotic events occurred post-splenectomy and one occurred under thrombopoietin-receptor agonist therapy.