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The primary aim of this study was to describe the use of primary anti-infective prophylaxis (AP) in common clinical practice in patients affected by immune thrombocytopenia (ITP) and treated with RTX. Population studied consisted of patients affected by ITP (age ≥ 18 years) who had received at least one dose of RTX from January 2008 to June 2018. Five Italian haematology centres participated in the current study. Data were retrospectively collected: demographic data (age, gender), concomitant comorbidities and previous therapies for ITP, characteristics of AP, the occurrence of infections and their management. The ITP cohort consisted of 67 patients sub-grouped into two categories according to the administration of AP: (1) treated with AP (N= 34; 51%) and (2) not treated with AP (N=33, 49%). AP consisted of combined trimethoprim/sulfamethoxazole (TMP/SMX) and acyclovir (AC) in half of patients. TPM/SMX as a single agent was adopted in 32% patients and one patient received only AC. Overall, infections were experienced in 15% of patients during follow-up with a similar proportion in the 2 groups (treated and not treated) of patients (14.7% vs 15%). Clinical course of infections was however, less severe in patients treated with AP, where all infections were grade 2 and did not require hospitalization. In neither group of patients was reported Pneumocystis pneumonia. In conclusion, despite the absence of clear evidence, our analysis shows that AP in patients with ITP receiving RTX is frequently adopted, even if in the absence of well-defined criteria. Prophylaxis administration is quite consistent within the same haematological Center; thus, it seems related to clinicians' experience.

INTRODUCTION: Primary Immune thrombocytopenia (ITP) in the elderly is a major clinical challenge which is increasingly frequent due to global ageing population. MATERIALS AND METHODS: To describe baseline ITP features, management, and outcome, a centralized electronic database was established, including data of 451 patients aged ≥60years that were treated from 2000 onwards and were observed for ≥1year (total observation of 2704 patient-years). RESULTS: At ITP diagnosis, median age was 71.1years (age≥75: 42.8%); 237 (53.9%) patients presented with haemorrhages (grade≥3: 7.5%). First-line therapy included prednisone (82.9%), dexamethasone (14.6%), thrombopoietin-receptor agonists (TRAs, 1.3%), and oral immunosuppressive agents (1.1%). Prednisone starting dose ≥1mg/kg/d (p=.01) and dexamethasone 40mg/d (p&nbsp

Dear Editor, In the current clinical practice, minimal criteria to define thrombotic thrombocytopenic purpura (TTP) are the presence of signs of microangiopathic haemolytic anaemia and low platelet (PLT) count [1]. TTP relapses (20–50 % of cases) are defined as the recurrence of acute TTP symptoms 30 days after the first episode, while exacerbations occur within 30 days [2]. We here report on an atypical case of acquired TTP where minimal criteria were met only after many recurrent macrovascular ischemic events. A 42-year old Caucasian woman with a history of coronary and cerebral ischemic events was admitted on June 2013, following a recurrent transient ischemic attack (TIA). She had severe anaemia and thrombocytopenia with laboratory signs of intravascular haemolysis and mild renal impairment. In her past medical history, recurrent ischemic events occurred from 2008 to 2012, without known risk factors and regardless of treatment. The patient was treated with acetyl salicylic acid (100 mg daily) after the first ischemic event (stroke, in 2008) plus anticoagulation with warfarin after the third stroke (in 2010), with a good compliance . Screening for autoimmune disease, Factor V Leiden and Factor II (G20210A) gene mutations and circulating anticoagulants were normal. Blood cell count and peripheral blood smear did not show any significant abnormalities in the past, without evidence of schistocytes; a slight decrease in PLT count (92×109/L) was observed only once (in 2012), at that time, ADAMTS13 activity (ADAMTS-13: AC) was 65 %. At the current admission, ADAMTS-13: AC was 6 %, inhibitors were at high titer (2 Bethesda Units, BU). A genetic test for Upshaw-Schulman syndrome was normal. Therapeutic plasma exchange (TPE) and prednisone (1 mg/kg body weight) treatment was started and maintained until clinical and haematological remission [3]. TPE (Gambro®, Italy) was performed daily for 3 weeks with an increase of ADAMTS13: AC to 50 %. TTP exacerbated after 10 days, it was well controlled with weekly rituximab (375 mg/m body surface), for 4 weeks. A relapse occurred after 37 days with the patient presenting seizures: ADAMTS-13: AC, which was 54 % after the last treatment, had decreased to 6 %. A second course of weekly rituximab, followed by maintenance with azathioprine (50 mg twice daily for 10 days, then 50 mg, daily) was followed by clinical and haematological remission. Rituximab was re-administered for its previous rapid efficacy and to prevent further complications [4]. The patient is alive and free from recurrences for 18 months; azathioprine maintenance is still on course. ADAMTS13: AC is monitored every 4 months, and it ranges from 45 to 51 %; inhibitors are no longer detectable. * Alessandro Lucchesi alessandro.lucchesi@irst.emr.it