Factors predictive of leg-ulcer healing in sickle cell disease: a multicentre, prospective cohort study.

BACKGROUND: Leg ulcers (LUs) are a chronic and severe complication of sickle cell disease (SCD). A prospective study in patients with SCD to identify factors associated with complete healing and recurrence of LUs is lacking. OBJECTIVES: To determine clinical and biological factors associated with SCD-LU complete healing and recurrence. METHODS: This prospective, observational cohort study was conducted at two adult SCD referral-centre sites (2009-2015) and included 98 consecutive patients with at least one LU lasting ≥ 2 weeks. The primary end points compared patients with healed vs. nonhealed LUs at week 24, and patients with vs. without recurrence during follow-up. RESULTS: The median (interquartile range) LU area, duration and follow-up were, respectively, 6·2 cm2 (3-12·8), 9 weeks (4-26) and 65·8 weeks (23·8-122·1). At week 24, LUs were healed in 47% of patients, while 49% of LUs recurred. Univariate analyses identified inclusion LU area < 8 cm2 (82% vs. 35%; P < 0·001), inclusion LU duration < 9 weeks (65% vs. 35%; P = 0·0013) and high median fetal haemoglobin level (P = 0·008) as being significantly associated with complete healing at week 24, and low lactate dehydrogenase level (P = 0·038) as being associated with recurrence. Multivariate analyses retained LU area < 8 cm2 (odds ratio 6·73, 95% confidence interval 2·35-19. 31; P < 0·001) and < 9 weeks' duration (OR 3·19, 95% confidence interval 1·16-8·76; P = 0·024) as being independently associated with healing at week 24. Factors independently associated with recurrence could not be identified. CONCLUSIONS: SCD-LU complete healing is independently associated with the clinical characteristics of LUs rather than the clinical or biological characteristics of SCD.

[Symptomatic extramedullary haematopoiesis in ß-thalassemia: A retrospective single centre study]. / Hématopoïèse extra-médullaire symptomatique au cours des ß-thalassémies: étude rétrospective monocentrique.

INTRODUCTION: Symptomatic extramedullary hematopoiesis (EH) is a rare but potentially severe phenomenon which occurs in ß-thalassemia. There are no treatment guidelines. METHODS: Retrospective single centre study including the cases of symptomatic EH encountered between 1997 and 2014 in a unit specialised in red blood cell genetic disorders. Description of clinical, biological and radiological characteristics of the patients, treatments received, and outcomes. RESULTS: Among 182 ß-thalassemia patients followed during the study period, 7 cases of symptomatic EH were diagnosed. They were 5 men and 2 women, and their mean age was 37 years. Four patients were splenectomised, two patients were regularly transfused, and four patients had already received erythropoietin. EH was localised in intravertebral areas and responsible for dorsal spinal cord compression in 5 patients, in paravertebral dorsal area in 1 patient, and in presacral area in 1 patient. The mean hemoglobin level at diagnosis was 7.9 g/dL. Treatment administered included: red cell transfusion in 6 cases, associated with hydroxyurea in 5 cases and/or radiotherapy in 3 patients. One patient was treated with surgery and HU. After a median follow-up of 41 months, clinical recovery was complete in 2 patients and partial in 5 patients. CONCLUSION: EH must be suspected in ß-thalassemia in patients presenting clinical signs of organ compression, and a typical radiological aspect. The functional prognosis depends on the rapidity of treatment, which includes red blood cell transfusion, hydroxyurea, radiotherapy, and rarely surgery. Long-term outcome is uncertain.

[French guidelines for the management of adult sickle cell disease: 2015 update]. / Recommandations françaises de prise en charge de la drépanocytose de l'adulte: actualisation 2015.

Sickle cell disease is a systemic genetic disorder, causing many functional and tissular modifications. As the prevalence of patients with sickle cell disease increases gradually in France, every physician can be potentially involved in the care of these patients. Complications of sickle cell disease can be acute and chronic. Pain is the main symptom and should be treated quickly and aggressively. In order to reduce the fatality rate associated with acute chest syndrome, it must be detected and treated early. Chronic complications are one of the main concerns in adults and should be identified as early as possible in order to prevent end organ damage. Many organs can be involved, including bones, kidneys, eyes, lungs, etc. The indications for a specific treatment (blood transfusion or hydroxyurea) should be regularly discussed. Coordinated health care should be carefully organized to allow a regular follow-up near the living place and access to specialized departments. We present in this article the French guidelines for the sickle cell disease management in adulthood.

[Intermittent prolonged fever triggered by efforts]. / Fièvre prolongée intermittente survenant à l'effort.

INTRODUCTION: Fever of unknown origin is a common reason for care in internal medicine. The wide variety of possible etiologies makes it difficult to standardize the diagnostic work-up that has to be primarily guided by the interview and physical examination. CASE REPORT: We report a case of prolonged fever having as main characteristics to be intermittent and triggered by efforts. The diagnosis of cerebrospinal fluid shunt infection with Propionibacterium acnes was finally made. In reaching this conclusion, many tests were needed, including renal explorations with biopsy showing an aspect of shunt nephritis. CONCLUSION: Prolonged fever of unknown origin in a patient having prosthetic material should raise the suspicion of prosthesis infection (especially if the fever is associated with efforts).

[Antifungal combination therapy in invasive fungal infections]. / Associations antifongiques dans les infections fongiques invasives.

Invasive fungal infections (candidiasis, aspergillosis and cryptococcosis) are major complications in immunocompromised patients and account for high morbidity and mortality. Guidelines on first-line therapy of invasive fungal infections are based on randomised clinical trials or experts' guidelines and include essentially single antifungal therapies, except for cryptococcosis. However, the severe prognosis of these infections raises the interest of antifungal associations, in first line or second-line therapy, and many experimental data have been published on this issue. In humans, amphotericin B plus flucytosine combination therapy in cryptococcal meningitis has been largely validated, especially in HIV-infected patients. Also, a few studies demonstrated that the combination of fluconazole plus amphotericin B in candidiasis is not antagonistic and that caspofungin plus polyene or caspofungin plus azole combinations may be beneficial in invasive aspergillosis. Randomised studies are necessary to confirm these data. A study assessing anidulafungin plus voriconazole association in aspergillosis is oncoming.