Limbic encephalitis is a disease that can occur in childhood or adolescence with many of the hallmarks of the adult disorder, suggesting that both result from similar pathogenic processes. Since most of the cases were non-paraneoplastic, as now also recognised in adults, more systematic and aggressive immunotherapies should be evaluated in order to improve outcomes.
Ancestim (r-MetHuSCF) is available in France for compassionate use in patients who are candidates for high-dose chemotherapy and autologous transplantation, and who failed in previous attempts at mobilization and collection. We report here data from 513 adult patients who benefited from this program, between January 1998 and July 2007. Given with systematic premedication, ancestim was generally well tolerated, although severe but not life-threatening adverse events were reported in 12 individuals. Overall, a graft was obtained or completed for 235 patients (46%). The median number of collected CD34 þ cells was 3.00 Â 10 6 /kg (range: 0.03-39.50). The target threshold of 2 Â 10 6 CD34 þ cells/kg was reached in 161 patients (31%). Factors associated with collection were diagnosis of myeloma, no previous autologous transplant, no more than one previous failed attempt and a mobilization regimen including cytotoxic agents. A total of 207 patients (40%) proceeded to high-dose chemotherapy and autologous transplantation. The median time to reach 0.5 Â 10 9 /L neutrophils and 20 Â 10 9 /L platelets was 12 (6-40) and 13 (0-31) days, respectively. We conclude that a combination of ancestim with filgrastim successfully mobilized CD34 þ cells in peripheral blood, and allowed adequate collection in preparation for autologous transplantation in approximately one-third of poorly mobilizing patients.
Major sickle cell disease syndrome (SCD) is a set of potentially serious and disabling constitutional haemoglobin pathologies characterised by chronic haemolysis and vaso‐occlusion phenomena. If expression takes the form of acute vaso‐occlusive crisis, SCD is currently considered to be a chronic systemic pathology, primarily associated with vasculopathy and ischaemia‐reperfusion phenomena. The haemolytic aspect of the disease may be associated with endothelial dysfunctional complications, including leg ulcers, which are a classic spontaneous complication of major SCD. Their frequency, all aetiologies combined, varies considerably according to the series under consideration. Hydroxycarbamide has become the standard treatment for some SCD phenotypes, but has classically been described as one of the causes of leg ulcer. This causality is widely debated and is still difficult to establish because it is a specific complication of the disease. Comorbidity factors (eg, iron deficiency) are also often implicated as causal or aggravating factors so research into all the potential aetiologies of leg ulcers in a sickle cell patient must be exhaustive. We discuss the aetiologies of a leg ulcer in a patient treated by hydrocarbamide for major SCD. The imputation of the drug was established, followed by a marrow allograft in this patient.
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