A 63-year-old Caribbean man was admitted to the hospital for low back and pelvic pain. His medical history included sickle cell hemoglobin (Hb) C disease with no vasoocclusive crisis within the last 20 years, and chronic obstructive pulmonary disease treated with inhaled corticosteroids for the last 4 years. A few hours after admission, respiratory distress with polypnea occurred, requiring an oxygen flow rate of 12 L per minute. At that time, a thrombocytopenia was detected in peripheral blood, with a platelet count of 112,000/mm 3 , as well as increased hemolysis (LDH 4990 IU/L; normal range 125-250). He was transferred to the Intensive Care Unit. The patient's neurological and respiratory status got worse in the Intensive Care Unit, with agitation, confusion, and hypercapnic acidosis leading to his intubation. Disseminated intravascular coagulation, thrombocytopenia, and leukopenia developed. Chest computed tomography scan showed lung bilateral alveolar condensation in the posterior basal segment and bilateral pleural effusions. A bone marrow smear was performed and showed bone marrow necrosis. The diagnosis of pulmonary infection without bacterial identification was made; the patient required antibiotic therapy combination and blood transfusion. The wake-up after initial sedation was delayed and no real consciousness after 4 days without sedation was detected, therefore leading to further investigations. ASSESSMENT An electroencephalogram, a lumbar puncture, and a cerebral magnetic resonance imaging (MRI) scan were performed: Funding: None.
A number of studies have demonstrated that cytomegalovirus (CMV) viraemia is a strong predictor for CMV end-organ disease (EOD) and death in HIV-infected patients. We assess the efficacy and safety of pre-emptive anti-CMV therapy (PACT) for preventing these events. We performed a retrospective study of all HIV-infected patients seen in our institution who had detectable CMV viraemia in 2007. Seventy-one patients with advanced HIV disease (median CD4 cell count = 61 cells/mm(3)) were studied. Sixteen patients received PACT (mainly valganciclovir). Patients who received PACT had lower CD4 cell counts and higher blood CMV DNA levels. The cumulative incidence of CMV EOD and death at one year was 44% and 21% in patients with and without PACT, respectively (p = 0.013). Both PACT and high blood CMV DNA levels were significantly associated with CMV EOD and death in unadjusted analysis. In adjusted analyses, only blood CMV DNA levels remained significantly associated with the risk of CMV EOD and death, whereas PACT was associated with a non-significant trend towards reduced CMV EOD or death (hazard ratio: 0.25, p = 0.13). Five patients with PACT experienced severe drug-related adverse events. In conclusion, the use of PACT in HIV-infected patients with CMV viraemia could improve outcome but is associated with significant toxicity.
Summary
The role of mast cells has been questioned in sickle cell disease (SCD). We performed a prospective study evaluating plasma histamine and tryptase levels in a cohort of paediatric and adult patients, in steady state (n = 132) and during vaso‐occlusive crisis (VOC) (n = 121). Histamine level was elevated in 18% of patients in steady state and in 61% during VOC. Median histamine level was significantly higher during VOC than in steady state (24·1 [7·0–45·0] vs 9·6 [6·2–14·4] nmol/l, P < 0·0001). Tryptase level was slightly increased during VOC without reaching pathological values. These results suggest a role of mast cell activation in SCD pathophysiology.
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