Splenectomy and/or cyclophosphamide as salvage therapies in thrombotic thrombocytopenic purpura: the French TMA Reference Center experience: SALVAGE THERAPIES IN SEVERE TTP

BACKGROUND: The objective was to assess the efficacy and safety of splenectomy and cyclophosphamide as salvage therapies in severe thrombotic thrombocytopenic purpura (TTP).STUDY DESIGN AND METHODS: During a 10-year period, patients who did not improve with plasma exchanges, steroids, vincristine, and/or rituximab were considered for splenectomy or cyclophosphamide. Patients with a documented severe (<10% of normal value) acquired ADAMTS13 deficiency are reported here. RESULTS: Eighteen patients with a severe acquired ADAMTS13 deficiency required a salvage therapy. Thirteen patients had a splenectomy 19 (interquartile range [IQR], 10-51) days after TTP diagnosis. One patient died the day after splenectomy. The remaining patients improved platelets (PLTs) until Day 6, along with a rapid and major lactate dehydrogenase improvement. Six patients, however, subsequently experienced a transient worsening. Durable PLT count recovery in survivors was observed within 13 (IQR, 11.5-25.5) days. Postoperative complications included thromboembolic events (two cases) and infections (five cases). Five patients received pulses of cyclophosphamide 12 (IQR, 12-15) days after TTP diagnosis. All patients recovered PLTs 10 (IQR, 9-24) days after the first pulse and two experienced a transient worsening. Three patients experienced infections. Three relapses occurred 5 months, 2.5 years, and 4.5 years after splenectomy and one relapse occurred 3.5 years after cyclophosphamide. After a 2.5 (IQR, 0.75-6.2)-year follow-up, the overall survival was 94%. CONCLUSION: Cyclophosphamide and splenectomy provide comparable high remission rates in severe TTP with acceptable side effects and should be considered in the more severe patients who do not improve with other therapies

Conservative management of pancreatic fistula after pancreaticoduodenectomy with pancreaticogastrostomy.

BACKGROUND: Pancreatic fistula (PF), which is a major complication of pancreaticoduodenectomy (PD), can be treated conservatively or by reoperation. The aim of this study was to evaluate conservative management of PF, which was attempted whenever possible as a first-intention treatment in a large series of PD. STUDY DESIGN: From 1990 to 2000, 242 patients underwent PD with pancreaticogastrostomy. PF was observed in 31 (13%) and was defined by an amylase-rich surgical drainage fluid (above fivefold serum amylase) after postoperative day 5, or by presence on CT scan of a fluid collection located close to the anastomosis or containing amylase-rich fluid, or by operative findings in case of reoperation. Conservative management included total parenteral nutrition, nasogastric suction, imaging-guided percutaneous drainage of collection when necessary, and somatostatin or its analogues. RESULTS: PF was symptomatic in 20 patients (65%). Amylase level on surgical drainage fluid was elevated in 23 patients (74%). Four patients (13%), including two with hemorrhage and two with intraabdominal collection not accessible by percutaneous approach, were not considered for conservative management and underwent early reoperation. Conservative management was successful in the 27 patients (100%) in whom it was attempted, including the 10 who required percutaneous drainage. The only death (3%) occurred after massive hemorrhage complicating misdiagnosed PF. Mean hospital stay was 36 +/- 12 days (range 18 to 71) after successful conservative management. CONCLUSIONS: Conservative management of PF complicating PD is feasible and successful in above 85% of patients

Fistula plug in fistulising ano-perineal Crohn's disease: a randomised controlled trial

International audienceBackground and aims: Anal fistula plug (AFP) is a bioabsorbable bioprosthesis used in ano-perineal fistula treatment. We aimed to assess efficacy and safety of AFP in fistulising ano-perineal Crohn's disease (FAP-CD). Methods: In a multicentre, open-label, randomized controlled trial we compared seton removal alone (control group) with AFP insertion (AFP group) in 106 Crohn's disease patients with non or mildly active disease having at least one ano-perineal fistula tract drained for more than one month. Patients with abscess (collection ≥ 3 mm on MRI) or recto-vaginal fistulas were excluded. Randomization was stratified in simple or complex fistulas according to AGA classification. Primary end point was fistula closure at week 12.Results54 patients were randomized to AFP group (control group 52). Median fistula duration was 23 [10-53] months. Median Crohn's Disease Activity Index at baseline was 81 [45-135]. Fistula closure at week 12 was achieved in 31.5% patients in AFP group and in 23.1 % in control group (relative risk stratified on AGA classification, RR: 1.31; 95%CI: 0.59-4.02; p=0.19). No interaction in treatment effect with complexity stratum was found. 33.3% patients with complex fistula and 30.8% patients with simple fistula closed the tracts after AFP, as compared to 15.4% and 25.6% in controls respectively (RR of success=2.17 in complex fistula vs. RR=1.20 in simple fistula; p= 0.45). Concerning safety, at week 12, 17 patients developed at least one adverse event in AFP group vs. 8 in controls (p=0.07). Conclusion: AFP is not more effective than seton removal alone to achieve FAP-CD closur