Impact of prenatal corticosteroid therapy on sickle cell disease in pregnant women.

OBJECTIVE: To evaluate safety of prenatal corticosteroids in pregnancies of women with sickle cell disease. METHODS: A multicenter observational study of patients with sickle cell disease, comparing vaso-occlusive crises (VOC) requiring hospital care between pregnancies with versus without prenatal corticosteroids. RESULTS: In 40 pregnancies exposed to prenatal corticosteroids, compared with 370 unexposed pregnancies, VOC were not more frequent (62.5% vs 57.9%, P = 0.578) but they were more severe, with more intensive care hospitalizations (25.0% vs 12.9%, P = 0.039), emergency transfusions (44.7% vs 22.7%, P = 0.006), and acute chest syndromes (22.5% vs 8.9%, P = 0.010). These differences persisted after adjustment for severity and type of sickle cell syndrome (for intensive care admission adjusted odds ratio [aOR] 2.73, 95% confidence interval [CI] 1.10-6.79, P = 0.031 and for acute chest syndrome aOR 4.15, 95% CI 1.57-14.4, P = 0.008). VOC occurred on average 1.2 days following steroid administration. When comparing 36 patients receiving corticosteroids for fetal maturation with 58 patients who were hospitalized for obstetrical complications before 34 weeks of pregnancy but that did not receive corticosteroids, VOC incidence was not significantly higher (41.7% vs 31.5%, P = 0.323). CONCLUSION: The present study was the first to study the impact of prenatal corticosteroids on sickle cell disease. They were associated with more severe VOC, suggesting that steroids should be avoided in these women.

Leg ulcer induced by hydroxycarbamide in sickle cell disease: What is the therapeutic impact?

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.

Extracorporeal chemophototherapy for the treatment of graft-versus-host disease: hematologic consequences of short-term, intensive courses.

BACKGROUND AND OBJECTIVES: Extracorporeal chemophototherapy (ECP) is considered an immunomodulatory agent useful in both acute and chronic graft-versus-host disease (GVHD). Little is known about the best treatment schedule, and there are no data concerning hematologic parameters and cellular compositions of products during the treatment. DESIGN AND METHODS: This was a single-center study of 27 patients treated with ECP for corticoresistant GVHD. Treatment was given in a short-term series of six courses over 3 weeks, and in case of response, consolidation treatment was given until complete response or stabilization of lesions. RESULTS: Nine out of 12 patients with acute GVHD responded to treatment. In patients with chronic GVHD, 13 out of 15 patients responded (11 complete and 2 partial responses). Responses were obtained essentially in skin or gut lesions; ECP was of particular effect in three cases of bronchiolitis obliterans associated with transplantation, with all three patients responding. Hematologic consequences were studied in patients with chronic GVHD: hemoglobin levels increased significantly after treatment and a reduction in red blood cell transfusion requirements was also observed. INTERPRETATION AND CONCLUSIONS: ECP is effective in both chronic and acute GVHD, particularly in lung forms. ECP can reduce the duration of immunosuppressive therapy and improve erythroid recovery. ECP product quality, including standardization for the number of mononuclear cells for each patient, needs further investigation.