Use of recombinant factor VIIa for emergency reversal of anticoagulation.

CONTEXT: There is limited data regarding the use of activated recombinant factor VII (rFVIIa) in anticoagulated patients requiring reversal. AIMS: To identify and describe characteristics of subjects who received rFVIIa as part of emergency treatment aimed at improving hemostasis. SETTINGS AND DESIGN: Data was obtained from an international peer-reviewed registry haemostasis.com. This registry contains data reported by physicians, who had elected to use rFVIIa to control bleeding in an emergency clinical situation. The contributors' approval for inclusion in the study was obtained and they were requested to validate and update information. MATERIALS AND METHODS: Database review of cases receiving rFVIIa to manage bleeding coherent with the use of anticoagulant therapy. STATISTICAL ANALYSIS: The Wilcoxon signed rank test was used to compare requirements for blood products and crystalloids/colloids during the 24h preceding and following rFVIIa administration, as well as changes in the levels of clotting factors during that period. RESULTS: Eighteen patients were treated with rFVIIa (median dose: 87.35 microg/kg; range: 20.0-106.0 microg/kg) for bleeding. Anticoagulants requiring reversal included low-molecular-weight heparin (n = 6), unfractionated heparin (n =8), coumarin (n =3) and warfarin (n=1). All patients had failed to respond to traditional antidotes and blood products. Following administration, bleeding stopped in 10, markedly decreased in five and slowed in the remaining three. Amongst 12/16 patients, a response was observed within 2.0 h of first administration. The requirement for blood products and crystalloids/colloids decreased ( P < 0.05) after rFVIIa administration. rFVIIa was well tolerated. CONCLUSIONS: rFVIIa may play a role in control of untoward bleeding in subjects receiving anticoagulation therapy.

Platelet function analysis in children with Schönlein-Henoch syndrome.

BACKGROUND: Schönlein-Henoch syndrome (SHS) or anaphylactic purpura in childhood is the result of pathologic and immunologic responses to different antigens. These antigens could induce the formation of immune complexes responsible for vasculitis and their precipitation on the endothelium of small blood vessels. Purpuric bruises, hematuria, hematemesis, melena, or hematochesis may suggest coagulation disturbances. Increasing bleeding tendency may suggest platelet function disturbance. To examine the qualitative function of platelets in children with SHS, we decided to analyze its aggregation function. METHODS: Using the Born method of testing, we analyzed platelet aggregation in 24 children with SHS. RESULTS: Based on the aggregograms examined, we observed that most patients had abnormal aggregation curves, in which platelets demonstrated a block of release of the endogenous ADP, with or without disaggregation. CONCLUSIONS: One clinical symptom of SHS appearing in most patients is a mild or increased tendency toward bleeding. On measuring induced aggregation of platelets in children with SHS, we observed that the qualitative function of platelets was disturbed.

Etoposide as the basic and interferon-alpha as the maintenance therapy for Langerhans cell histiocytosis: a RTC.

The treatment of patients who suffer from a disseminated form of Langerhans cell histiocytosis (LCH) is still controversial. So far, few larger randomized studies have been performed. The authors present 3 patients with a disseminated form of LCH--4 months, 9 months, and 2 years old, respectively. The lesional Langerhans cells in each patient showed positive immunohistochemical reaction to S-100 protein and the presence of Birbeck granules was confirmed by electron microscopy. All the patients were treated with etoposide (VP-16), 200 mg/m2 for 3 consecutive days, with 15 cycles at intervals of 3 weeks between each cycle, followed by maintenance therapy with IFN-alpha. All 3 patients reached complete stabile remission. The patients were young, at high risk, with multiple-organ involvement of LCH, and two of them had obvious signs of organ dysfunction at presentation, suggesting a poor prognosis. All remain disease-free several years after therapy. The results suggest that INF-alpha may prevent recurrences in high-risk patients.