Thromboembolic complications in autoimmune hemolytic anemia: Retrospective study.

INTRODUCTION: A small number of retrospective studies suggest AIHA to be associated with an increased risk to suffer from thromboembolic events. However, based on these studies it remains unclear whether the complement activation per is a risk factor to develop thromboembolic events in AIHA patients. The aim of this retrospective study is to investigate the incidence of thromboembolic events and the relation to complement activation in a cohort of AIHA patients. PATIENTS AND METHODS: We included 77 patients in this study with a positive DAT and hemolytic parameters or with AIHA diagnosis based on the medical report. The included patients were screened for thromboembolic events (TEE) and have been stratified in groups with and without complement activation based on the positivity for complement in the DAT. RESULTS: Of the 77 included patients, 51 (66%) had warm AIHA, 13 (17%) cold-AIHA, 5 (7%) mixed AIHA, and 8 (10%) atypical AIHA, respectively. Primary and secondary AIHA was diagnosed in 44% and 56%, respectively. Twenty patients (26%) suffered from TEE. The majority (80%) of these patients suffered from warm AIHA and 10% from cold-AIHA. Hemolysis parameters did not differ in patients with and without TEE. There was no correlation with complement activation as evidenced by a positivity for complement in the monospecific DAT with the occurrence of TEE. CONCLUSION: AIHA is associated with an increased risk of TEE. Based on these results prophylactic anticoagulation might be considered as soon as the diagnosis of AIHA is confirmed.

Adding bendamustine to melphalan before ASCT improves CR rate in myeloma vs. melphalan alone: A randomized phase-2 trial.

Definite cure remains exceptional in myeloma patients even after high-dose chemotherapy (HDCT) with melphalan (Mel) and autologous stem cell transplantation (ASCT). Thus, improving efficacy of HDCT in MM remains an unresolved issue. This randomized phase II trial compared standard 200 mg/m2 Mel HDCT to experimental HDCT with 200 mg/m2 bendamustine, given both at days -4 and -3, combined with 100 mg/m2 melphalan at days -2 and -1 (BenMel) before ASCT as first-line consolidation in myeloma patients. The primary endpoint aimed to identify at least a 15% improvement in the complete remission rate (stringent CR + CR) after HDCT with BenMel compared with Mel alone. A total of 120 MM patients were 1:1 randomized. The rate of sCR/CR after ASCT was higher in BenMel than in Mel treated patients (70.0% vs. 51.7%; p = 0.039). Three patients in the BenMel group (5.0%) had reversible acute renal insufficiency compared with none in Mel patients. Minimal residual disease negativity (<10-5) by flow cytometry was observed in 26 (45.6%) BenMel patients and 22 (37.9%) in the Mel group (p = 0.375). Our data suggest that BenMel HDCT is safe and improves the sCR/CR rate compared with standard Mel alone.

Acquired factor XIII deficiency: a therapeutic challenge.

Less than 60 cases of acquired factor (F)XIII deficiencies have been reported, most having distinct clinical features. To illustrate the therapeutic challenges of acquired FXIII inhibitors, we report a case of a 65-year-old patient with no previous bleeding history who suddenly developed massive haemorrhages associated to a strong and isolated FXIII inhibitor. No underlying disorder has been detected till now after three years of follow-up. Despite aggressive treatment with prednisone, rituximab, cyclophosphamide, immunoglobulin, immunoadsorption and immune tolerance his inhibitor is still present, although at low titre and with a clinical benefit since the patient has no more bleed since more than one year. Moreover the patient had a venous thromboembolic complication. After a review of the management of acquired FXIII deficiency patients and based on the management of acquired haemophilia we discuss a possible strategy for such difficult cases.