Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy which was first included as an independent cutaneous lymphoma in the 2008 World Health Organisation (WHO) ...classification (1). BPDCN usually has an extremely poor prognosis, with quick relapses after chemotherapy (2; 3). Here, we report two cases of patients diagnosed in 2011 with BPDCN and myelodysplasia, and who were treated for the first time with 5‐azacytidine (5‐Aza); a drug approved by the Food and Drug Administration (FDA) and mainly used in the treatment of myelodysplastic syndrome (Kaminskas E, et al. 2005 Clin Cancer Res, 11, 3604–8). The first case was an 81‐year‐old man who presented with unusual CD10+, CD56‐ immunohistochemistry and 45X, ‐Y abnormality using fluorescent in situ hybridization (FISH) analysis. The second case was a 78‐year‐old woman who manifested monosomy 13 and chromosome instability due to D13S319 locus deletion in 13q14 as determined by FISH. Both patients showed excellent responses of their skin lesions after one cycle of chemotherapy, and their hematological disease was stabilized; however, pulmonary sepsis set in, followed by neutropenia after the fourth and the fifth cycle of treatment, that is, eight and 9 months postdiagnosis, respectively, leading to patient death.
Dietary supplementation with marine omega-3 polyunsaturated fatty acids (n-3 PUFA) can have beneficial effects on a number of risk factors for cardiovascular disease (CVD). We compared the effects of ...two n-3 PUFA rich food supplements (freeze-dried
Odontella aurita
and fish oil) on risk factors for CVD. Male rats were randomly divided into four groups of six animals each and fed with the following diets: control group (C) received a standard diet containing 7 % lipids; second group (HF high fat) was fed with a high-fat diet containing 40 % lipids; third group (HFFO high fat+fish oil) was fed with the high-fat diet supplemented with 0.5 % fish oil; and fourth group (HFOA high fat+
O. aurita
) received the high-fat diet supplemented with 12 % of freeze-dried
O. aurita
. After 8 weeks rats fed with the high-fat diet supplemented with
O. aurita
displayed a significantly lower bodyweight than those in the other groups. Both the microalga and the fish oil significantly reduced insulinemia and serum lipid levels.
O. aurita
was more effective than the fish oil in reducing hepatic triacyglycerol levels and in preventing high-fat diet-induced steatosis.
O. aurita
and fish oil also reduced platelet aggregation and oxidative status induced by high fat intake. After an OA supplementation, the adipocytes in the HFOA group were smaller than those in the HF group. Freeze-dried
O. aurita
showed similar or even greater biological effects than the fish oil. This could be explained by a potential effect of the n-3 PUFA but also other bioactive compounds of the microalgae.
Dietary changes are a major factor in determining cardiovascular risk. n-3 polyunsaturated fatty acids modulate the risk factors for metabolic syndrome via multiple mechanisms, including the ...regulation of the lipid metabolism. We therefore investigated the effect of Odontella aurita, a microalga rich in EPA, which is already used as a food supplement, on the risk factors for high-fat diet induced metabolic syndrome in rats.
Male Wistar rats were divided into 4 groups and were fed with a standard diet (control); with the standard diet supplemented with 3% freeze-dried O. aurita (COA); with a high-fat diet (HF); or with the high-fat diet supplemented with 3% of freeze-dried O. aurita (HFOA) for 7 weeks. In this study we evaluated the impact of these different diets on the risk factors for metabolic syndrome, such as hyperlipidemia, platelet aggregation, thromboxane B2 production, and oxidative stress.
After 7 weeks of treatment, high fat feeding had increased final body weight, glycemia, triacylglycerol, and total cholesterol levels in plasma and liver compared to the control diet. Collagen-induced platelet aggregation and basal platelet thromboxane B2 were also higher in the high-fat fed rats than in those in the control group. In the liver, oxidative stress was greater in the HF group than in the control group. O. aurita intake in HFOA-fed rats resulted in lower glycemia and lipid levels in the plasma and liver relative than in the HF group. Thus, in the HFOA group, n-3 polyunsaturated fatty acid levels in the tissues studied (plasma, liver, and platelets) were higher than in the HF group. Platelet hyper-aggregability tended to decrease in HFOA-fed rats as basal platelet thromboxane B2 production decreased. Finally, O. aurita reduced oxidative stress in the liver, with lower malondialdehyde levels and increased glutathione peroxidase activity.
O. aurita is a marine diatom rich in EPA as well as in other bioactive molecules, such as pigments. The synergistic effect of these microalgal compounds, displayed a beneficial effect in reducing the risk factors for high-fat induced metabolic syndrome: hyperlipidemia, platelet aggregation, and oxidative stress.
Replacement therapy with plasma‐derived or recombinant FVIII and FIX (pdFVIII/pdFIX or rFVIII/rFIX) concentrates is the standard of treatment in patients with haemophilia A and B, respectively. ...Measurement of factor VIII (FVIII:C) or factor IX (FIX:C) levels can be done by one‐stage clotting assay (OSA) or chromogenic substrate assay (CSA). The French study group on the Biology of Hemorrhagic Diseases (a collaborative group of the GFHT and MHEMO network) presents a literature review and proposals for the monitoring of FVIII:C and FIX:C levels in treated haemophilia A and B patients, respectively. The use of CSA is recommended for the monitoring of patients treated with pdFVIII or rFVIII including extended half‐life (EHL) rFVIII. Except for rFVIII‐Fc, great caution is required when measuring FVIII:C levels by OSA in patients substituted by EHL‐rFVIII. The OSA is recommended for the monitoring of patients treated with pdFIX or rFIX. Large discordances in the FIX:C levels measured for extended half‐life rFIX (EHL‐rFIX), depending on the method and reagents used, must lead to great attention when OSA is used for measuring FIX:C levels in patients substituted by EHL‐rFIX. Data of most of recent studies, obtained with spiked plasmas, deserve to be confirmed in plasma samples of treated patients.
Key Clinical Message
A patient with a marginal zone lymphoma received RCHOP and obtained PR. He received RDHAP, autograft, and obtained CR. Three months later, he developed Kaposi's sarcoma with ...spontaneous regression. Two months later, he developed DLBCL treated with R‐MIV with CR. Thereafter, he developed AML and died a few days later.
A patient with a marginal zone lymphoma received RCHOP and obtained PR. He received RDHAP, autograft, and obtained CR. Three months later, he developed Kaposi's sarcoma with spontaneous regression. Two months later, he developed DLBCL treated with R‐MIV with CR. Thereafter, he developed AML and died a few days later.
We evaluated the efficacy and safety of rituximab for the treatment of 23 elderly patients (median age 78 years) with warm autoimmune haemolytic anaemia (AIHA). The median follow-up was 31 months. ...Patients had received one to five previous treatments. Rituximab was administered by intravenous infusion at a dose of 375 mg/m
2
once weekly for 4 weeks. The OR rate was 86.9 % (CR = 39.1 %, PR = 47.8 %). Median OS was 87 months. The median OS of patients who reached CR could not be calculated, and that of patients with PR was 67 months. At last follow-up, eight of the 20 responding patients, including one patient in CR and seven in PR, had relapsed after a median of 6 months. Failure to achieve CR was a risk factor for relapse (
p
= 0.028). We did not identify any pretreatment characteristics predictive of response to rituximab. In conclusion, rituximab is an effective treatment for elderly patients with refractory warm AIHA.
•Argan oil and fish oil are commonly used as food supplements.•Their effects were tested on some risk factors for cardiovascular diseases in rats developing dietary-induced dyslipidemia.•Fish oil ...intake decreases plasma and liver lipid levels, and prevents adiposity development and prothrombotic effects in rats.•Argan oil supplementation does not affect adiposity or liver lipid levels, but decreases plasma lipid levels and improves oxidative status and platelet activity.•Fish oil and, to a less degree, argan oil thus represent promising nutritional tools in the prevention of cardiovascular diseases.
The aim of this study was to investigate the effects of two different sources of polyunsaturated fatty acid—fish oil (FO) and argan oil (AO)—on some risk factors for cardiovascular disease, such as platelet aggregation, dyslipidemia, and oxidative stress.
To explore this, four groups of six male rats were fed with different diets: The first group received a standard diet (control); the second group received a high-fat diet; the third was fed with a high-fat diet supplemented with 5% FO, and the last group received a high-fat diet supplemented with 5% AO.
After 8 wk of the diet, AO showed a decrease in plasma lipids similar to that of FO. However, unlike FO, AO had no significant effect on hepatic lipid levels. On the other hand, supplementation with AO and FO similarly reduced platelet hyperactivity induced by high-fat diet. Concerning the results of oxidative stress, AO showed an antioxidant effect in the tissues and platelets greater than that observed in the high-fat FO group.
For rats, the consumption of FO prevented the development of adiposity, restored insulin sensitivity, decreased plasma and liver lipid levels, and also prevented the prothrombotic effect. Intake of AO as a food supplement did not affect adiposity or liver lipid levels but decreased plasma lipid levels and improved oxidative status and platelet activity. FO and, to a lesser degree, AO thus represent promising nutritional tools in the prevention of cardiovascular disease.
Summary
Acquired factor V inhibitor (AFVI) is an extremely rare disorder that may cause severe bleeding. To identify factors associated with bleeding risk in AFVI patients, a national, multicentre, ...retrospective study was made including all AFVI patients followed in 21 centres in France between 1988 and 2015. All patients had an isolated factor V (FV) deficiency <50% associated with inhibitor activity. Patients with constitutional FV deficiency and other causes of acquired coagulation FV deficiencies were excluded. The primary outcome was incident bleeding and factors associated with the primary outcome were identified. Thirty‐eight (74 36–100 years, 42·1% females) patients with AFVI were analysed. Bleeding was reported in 18 (47·4%) patients at diagnosis and in three (7·9%) during follow‐up (7 0·2–48.7 months). At diagnosis, FV was <10% in 31 (81·6%) patients. Bleeding at diagnosis was associated with a prolonged prothrombin time that strongly correlated with the AFVI level measured in plasma {r = 0·63, 95% confidence interval (CI) 0·36–0·80, P < 0·05}. Bleeding onset during follow‐up was associated with a slow AFVI clearance (P < 0·001). The corresponding receiver operating characteristics curve showed that AFVI clearance was predictive of bleeding onset with an AFVI clearance of seven months with a sensitivity of 100% (95% CI: 29–100) and a specificity of 86% (95% CI: 57–98, P = 0·02). Kaplan–Meier analysis showed that AFVI clearance >7 months increased the risk of bleeding by 8 (95% CI: 0·67–97, P = 0·075). Prothrombin time at diagnosis and time for clearance of FV inhibitor during follow‐up are both associated with bleeding in patients with AFVI.
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