Recommandations pratiques de prise en charge de la drépanocytose de l’adulte

Lionnet, François; Arlet, J; Bartolucci, Pablo; Habibi, Anoosha; Ribeil, Jean‐Antoine; Stankovic, K.

doi:10.1016/j.revmed.2009.07.001

Cited by 47 publications

(16 citation statements)

References 26 publications

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“…To assure the reliability of specimen sampling, storage and analysis, patients arriving at the Emergency Department after midnight were included the next morning except weekends, and hospitalized in the Internal Medicine or Clinical Immunology Department specialized in SCD management (after January 2008). Transfusion indications according to French guidelines (Lionnet et al, 2009) were: anemia < 6/dL with symptoms, VOC lasting > 8 days without improvement, every acute severe complication, overt stroke, severe infection, multiorgan involvement, acute priapism lasting > 3 h and severe ACS. The latter was defined by one of the following: respiratory frequency > 30/min or < 10/min not related to opioid overdose, shallow breathing, breathlessness on speaking, impaired consciousness, important extension of auscultatory abnormality and/or radiological infiltrate, arterial blood–gas PO 2 < 60 mm Hg.…”

Section: Methodsmentioning

confidence: 99%

Score Predicting Acute Chest Syndrome During Vaso-occlusive Crises in Adult Sickle-cell Disease Patients

et al. 2016

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BackgroundVaso-occlusive crisis (VOC), hallmark of sickle-cell disease (SCD), is the first cause of patients' Emergency-Room admissions and hospitalizations. Acute chest syndrome (ACS), a life-threatening complication, can occur during VOC, be fatal and prolong hospitalization. No predictive factor identifies VOC patients who will develop secondary ACS.MethodsThis prospective, monocenter, observational study on SS/S-β0thalassemia SCD adults aimed to identify parameters predicting ACS at Emergency-Department arrival. The primary endpoint was ACS onset within 15 days of admission. Secondary endpoints were hospitalization duration, morphine consumption, pain evaluation, blood transfusion(s) (BT(s)), requiring intensive care and mortality.FindingsAmong 250 VOCs included, 247 were analyzed. Forty-four (17.8%) ACSs occurred within 15 (median [IQR] 3 [2, 3]) days post-admission based on auscultation abnormalities; missing chest radiographs excluded three patients. Comparing ACS to VOC, respectively, median hospital stay was longer 9 [7–11] vs 4 [3–7] days (p < 0.0001), 7/41 (17%) vs 1/203 (0.5%) required intensive care (p < 0.0001), and 20/41 (48.7%) vs 6/203 (3%) required BTs (p < 0.0001). No patient died. The multivariate model retained reticulocyte and leukocyte counts, and spine and/or pelvis pain as being independently associated with ACS; the resulting ACS-predictive score's area under the ROC was 0.840 [95% CI 0.780–0.900], 98.8% negative-predictive value and 39.5% positive-predictive value for the real ACS incidence.InterpretationThe ACS-predictive score is simple, easily applied and could change VOC management and therapeutic perspectives. Assessed ACS risk could lead to earlier discharges or close monitoring and rapid medical intensification to prevent ACS.

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Section: Methodsmentioning

confidence: 99%

Score Predicting Acute Chest Syndrome During Vaso-occlusive Crises in Adult Sickle-cell Disease Patients

et al. 2016

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“…European origin and patients of African ancestry, thereby explaining why DHTR is much more frequently observed in SCD than other hematological diseases. 15,16 However, DHTR mechanisms in SCD are not very well understood, and beyond the presence of alloAbs, and subsequent RBC destruction through phagocytosis and complement-mediated hemolysis,…”

Section: Introductionmentioning

confidence: 99%

Delayed hemolytic transfusion reaction in adult sickle‐cell disease: presentations, outcomes, and treatments of 99 referral center episodes

et al. 2016

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Delayed hemolytic transfusion reaction (DHTR) is one of the most feared complications of sickle-cell disease (SCD). We retrospectively analyzed the clinical and biological features, treatments and outcomes of 99 DHTRs occurring in 69 referral center patients over 12 years. The first clinical signs appeared a median of 9.4 [IQR, 3-22] days after the triggering transfusion (TT). The most frequent DHTR-related clinical manifestation was dark urine/hemoglobinuria (94%). Most patients (89%) had a painful vaso-occlusive crisis and 50% developed a secondary acute chest syndrome (ACS). The median [IQR] hemoglobin-concentration nadir was 5.5 [4.5-6.3] g/dL and LDH peak was 1335 [798-2086] IU/L. Overall mortality was 6%. None of the patients had been receiving chronic transfusions. Among these DHTRs, 61% were developed in previously immunized patients, 28% in patients with prior DHTR. Among Abs detected after the TT in 62% of the episodes, half are classically considered potentially harmful. No association could be established between clinical severity and immunohematological profile and/or the type and specificity of Abs detected after the TT. Management consisted of supportive care alone (53%) or with adjunctive measures (47%), including recombinant erythropoietin and sometimes rituximab and/or immunosuppressants. Additional transfusions were either ineffective or worsened hemolysis. In some cases, severe intravascular hemolysis can be likely responsible for the vascular reaction and high rates of ACS, pulmonary hypertension and (multi)organ failure. In conclusion, clinicians and patients must recognize early DHTR signs to avoid additional transfusions. For patients with a history of RBC immunization or DHTR, transfusion indications should be restricted. Am. J. Hematol. 91:989-994, 2016. © 2016 Wiley Periodicals, Inc.

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“…In concordance with these findings, the majority of survey respondents did not routinely transfuse for uncomplicated pregnancy. Published recommendations do not identify acute painful episodes without complications as an indication for transfusion [10, 12, 14]. In our survey, 9% of the sample indicated this was “always” an indication, whereas the combination of rarely and never constituted 50% of the responses.…”

Section: Discussionmentioning

confidence: 65%

“…The following differences were observed for indications, where there is greater consensus in the literature [8–12, 14]. Pediatricians “rarely” considered acute painful episodes an indication for transfusion in comparison to adult practice respondents (78% versus 26%) ( P < 0.0001).…”

Section: Resultsmentioning

confidence: 99%

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Transfusion Practices in the Management of Sickle Cell Disease: A Survey of Florida Hematologists/Oncologists

et al. 2012

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The purpose of this study was to characterize transfusion practices in the management of sickle cell disease and to identify factors attributing to differences in prescribing practices among Florida hematologists/oncologists. A cross-sectional study was performed in 2005-2006 utilizing a mail survey. The survey instrument addressed practice characteristics, sickle cell patient populations, transfusion settings, indications and techniques, red blood cell phenotype specifications/modifications, use of practice guidelines, and educational resource utilization. One hundred fifty two physicians (75% adult-oriented, 25% pediatric) completed the survey. Non-academic practice settings (78 %) were the primary location. Pediatric practices had a larger percentage of patients with overt strokes, and receiving hydroxyurea therapy than adult-oriented practices. The majority of survey respondents did not request limited phenotypically matched red blood cells on a routine basis. The majority of pediatric practices (60%) had individually defined transfusion practice guidelines in contrast to 8% of adult-oriented practices. There were statistically significant differences for pediatric and adult-oriented practices in managing certain acute and chronic transfusion indications. Analysis of clinical vignette data revealed variation among hematologists/oncologists in the transfusion management of common clinical scenarios. The study underscores the need for the development and dissemination of comprehensive sickle cell transfusion guidelines and protocols.

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Recommandations pratiques de prise en charge de la drépanocytose de l’adulte

Cited by 47 publications

References 26 publications

Score Predicting Acute Chest Syndrome During Vaso-occlusive Crises in Adult Sickle-cell Disease Patients

Score Predicting Acute Chest Syndrome During Vaso-occlusive Crises in Adult Sickle-cell Disease Patients

Delayed hemolytic transfusion reaction in adult sickle‐cell disease: presentations, outcomes, and treatments of 99 referral center episodes

Transfusion Practices in the Management of Sickle Cell Disease: A Survey of Florida Hematologists/Oncologists

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