Does glucose-6-phosphate dehydrogenase deficiency worsen the clinical features of sickle cell disease? A multi-hospital-based cross-sectional study.

peer reviewed[en] BACKGROUND: The impact of glucose-6-phosphate dehydrogenase deficiency(G-6-PD) on the clinical course of sickle cell disease(SCD) is still controversial. The objectives of this study were to determine the prevalence of G-6-PD deficiency in patients with SCD and its effect on their clinical course. METHODS: A cross-sectional study of 122 SCD patients and 211 healthy blood donors was conducted in Kisangani city. Data were collected through clinical examination supplemented by patient medical records, and laboratory tests based on a survey form. G-6-PD activity was measured by spectrophotometry and the screening for SCD by the HemoTypeSC® rapid test. Statistical analysis was done using SPSS ver. 20.0. RESULTS: The prevalence of G-6-PD deficiency did not differ between SCD and non-SCD subjects, 35.2% vs. 33.6% respectively(p = .767). When comparing the hemoglobin level between SCD patients with and without G-6-PD deficiency, no significant difference was observed. However, in the 6 months prior to the study, SCD patients with G-6-PD deficiency had on average more transfusions than non-deficient SCD patients, 0.64 ± 0.897 vs. 0.24 ± 0.486(p = .004). Similarly, considering the clinical events of the last 12 months prior to the study, there were more hospitalizations, major vaso-occlusive crises and anemia requiring blood transfusion among G-6-PD deficient SCD patients compared to no-deficient, respectively 1.42 ± 1.451vs. 0.76 ± 1.112(p = .007); 1.37 ± 1.092 vs. 0.85 ± 1.014(p = .005); 0.74 ± 0.902 vs. 0.38 ± 0.739 (p = .007). CONCLUSION: The prevalence of G-6-PD deficiency in SCD patients was high but did not differ from that observed in controls. In addition, G-6-PD deficiency appeared to worsen the clinical features of SCD. Nevertheless, prospective studies further clarifying this observation are needed

Eltrombopag for myelodysplastic syndromes or chronic myelomonocytic leukaemia with no excess blasts and thrombocytopenia: a French multicentre retrospective real-life study.

peer reviewedDespite a moderate prevalence in low-risk myelodysplastic syndromes (MDS) and chronic myelomonocytic leukaemia (CMML), thrombocytopenia remains a risk of severe bleeding and therapeutic options are still limited. There are only a few studies with eltrombopag (ELT), a thrombopoietin receptor agonist, in those patients. In this retrospective multicentre study, ELT was used in 50 patients with MDS and 11 with CMML, with no excess of marrow blasts and platelet counts of <50 × 109 /l in a 'real-life' situation. Platelet response occurred in 47 (77%) patients. The median (range) duration of response was 8 (0-69) months. None of the eight still responders who discontinued ELT had relapsed, at a median (range) of 16 (6-23) months after ELT discontinuation. Although 36% of the patients were anti-coagulated or anti-aggregated only 10% of patients had Grade ≥3 bleeding events. Thrombotic events were observed in six (10%) patients, who all but one had a medical history of arterial or venous thrombosis. Progression to acute myeloid leukaemia occurred in four (7%) patients. In this first 'real-life' study, ELT was effective and generally well tolerated in patients with MDS/CMML without excess blasts

Les anémies réfractaires

Refractory anemia, also known as myelodysplastic syndromes, forms a group of clonal diseases characterized by cytopenias with mostly rich bone marrow. Preferentially reaching an older population, the prognosis depends on both comorbidities and characteristics of the disease, which have been grouped into a score established in 1997 (" IPSS = International Prognostic Scoring System ") and revised in 2012 (" R-IPSS = Revised IPSS "). Overall survival and risk of transformation into acute nonlymphoblastic leukemia can now be estimated fairly accurately. Based on these characteristics, the treatment will be mainly supportive or will use several new molecules :growth factors, lenalidomide, 5-azacitidine, etc. A minority of patients may also benefit from allogeneic BMT or sometimes immunosuppressive therapy.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Les anémies réfractaires

Les anémies réfractaires, encore appelées syndromes myélodysplasiques, forment un ensemble de maladies clonales caractérisées par des cytopénies avec le plus souvent une moelle riche. Atteignant préférentiellement une population âgée, le pronostic dépend à la fois des comorbidités présentées et des caractéristiques propres de l’affection, lesquelles ont été regroupées en un score établi en 1997 (“ IPSS = International Prognostic Scoring System ”) et revu en 2012 (“ R-IPSS = Revised IPSS ”). La survie globale et le risque de transformation en leucémie aiguë non lymphoblastique peuvent dès lors être estimés de manière relativement précise. En fonction de ces caractéristiques, le traitement restera principalement supportif ou fera appel à certaines molécules dont notre arsenal thérapeutique s’est enrichi ces dernières années : facteurs de croissance, lénalidomide, 5-azacytidine, etc. Une minorité de patients pourra en outre bénéficier d’une greffe de moelle allogène ou parfois aussi d’un traitement immunosuppresseur

Importance of a medical treatment in mesenteric vein thrombosis (MVT)

Mesenteric vein thrombosis (MVT) and particularly superior mesenteric vein thrombosis (SMVT) can induce 5 to 15 percent of mesenteric and intestinal infarctions in a small and large bowels. The thrombotic process can be idiopathic or consecutive to inherited or acquired thrombophilic states. The clinical diagnosis of this event remains difficult and requires always specific imaging investigation to treat as soon as possible. Its evolution and mortality rate are quite different than these observed in arterial mesenteric ischemic accident. Medical treatment with thrombolytic, anticoagulant, antiplatelet and antispasmodic agents, initiated promptly after precocious diagnosis is able not only to prevent surgical procedure but also to reduce significantly the mortality and recurrence rate of this venous thrombotic event

Auto-immune acquired hemophilia A associated with clopidogrel: A treatable life-threatening condition

info:eu-repo/semantics/publishe

Sweet's syndrome induced by pegfilgrastim during a myelodysplastic syndrome AREB2: A case report

Le syndrome de Sweet est une dermatose neutrophilique aiguë caractérisée par l’apparition brutale de lésions cutanées accompagnées de fièvre, d’arthralgies, d’une leucocytose et d’une infiltration neutrophilique diffuse du derme, ainsi que par une excellente réponse à la corticothérapie. Observation Un patient de 46 ans, ayant un syndrome myélodysplasique, était hospitalisé pour une chimiothérapie. Au 8e jour de la chimiothérapie, il recevait une dose unique de pegfilgrastim. Trois jours plus tard, il développait de la fièvre, une conjonctivite, des arthralgies et des lésions papulonodulaires érythémateuses et douloureuses. Une antibiothérapie empirique à large spectre était débutée mais le patient se dégradait. La biologie montrait une pancytopénie et un syndrome inflammatoire. Les bilans microbiologique, auto-immun et le scanner thoracique revenaient négatifs. La biopsie cutanée était compatible avec un syndrome de Sweet. Un diagnostic de syndrome de Sweet sur pegfilgrastim était retenu et une corticothérapie intraveineuse était débutée avec une guérison rapide. Conclusion La survenue d’un syndrome de Sweet est une complication rare des traitements par G-CSF

Microvesicles and cancer

Microvesicles (MV) are since quite recently recognized as forming a unique network between cells. These very little fragments (<1 µm size) are actively released from their parent cells and are able to transfer both cellular and nuclear material. Although active debate remains on how to best detect MV, rendering some results questionable, high MV levels have been reported in aggressive tumours and have been correlated with a poor clinical outcome. Some tumour cell derived MV exhibit strong tissue factor dependent procoagulant activity. Their detection could actually predict the thrombotic risk in selected cancer patients. A growing body of evidence suggests cell microvesicles to be a major link between cancer and thrombosis. Current knowledge on MV in cancer will be reviewed here.http://www.ariez.nl/DownloadFile.lynkx?guid=cf294b72-1c86-45f5-a006-522bfc09dce8info:eu-repo/semantics/publishe