Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich's syndrome): frequency, clinical implication and prognosis

BACKGROUND: Episodic Angioedema with eosinophilia (EAE, Gleich\u27s syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M. METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France. RESULTS: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone. CONCLUSION: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare

Antiretroviral-naive and -treated HIV-1 patients can harbour more resistant viruses in CSF than in plasma

Objectives The neurological disorders in HIV-1-infected patients remain prevalent. The HIV-1 resistance in plasma and CSF was compared in patients with neurological disorders in a multicentre study. Methods Blood and CSF samples were collected at time of neurological disorders for 244 patients. The viral loads were >50 copies/mL in both compartments and bulk genotypic tests were realized. Results On 244 patients, 89 and 155 were antiretroviral (ARV) naive and ARV treated, respectively. In ARV-naive patients, detection of mutations in CSF and not in plasma were reported for the reverse transcriptase (RT) gene in 2/89 patients (2.2%) and for the protease gene in 1/89 patients (1.1%). In ARV-treated patients, 19/152 (12.5%) patients had HIV-1 mutations only in the CSF for the RT gene and 30/151 (19.8%) for the protease gene. Two mutations appeared statistically more prevalent in the CSF than in plasma: M41L (P = 0.0455) and T215Y (P = 0.0455). Conclusions In most cases, resistance mutations were present and similar in both studied compartments. However, in 3.4% of ARV-naive and 8.8% of ARV-treated patients, the virus was more resistant in CSF than in plasma. These results support the need for genotypic resistance testing when lumbar puncture is performe

Changes in epidemiology of leptospirosis in 2003–2004, a two El Niño Southern Oscillation period, Guadeloupe archipelago, French West Indies

Our study aimed at analysing the changes in epidemiological features of leptospirosis cases from the hospital of Pointe à Pitre in Guadeloupe in 2003–2004 compared to reliable data in 1994–2001. Leptospirosis incidence increased fourfold during 2002–2004, a period with two El Niño events. Whereas the main risk factors were unchanged (male gender, occupational exposure, contact with cattle or pigs) a major role of rodent exposure emerged (52%, P=0·02, multivariate analysis). Interestingly, mean age of cases shifted to the older population (51·7 years vs. 43 years, P<0·05). Moreover, the Ballum serogroup rose dramatically (36% of incidence) competing with the Icterohaemorragiae serogroup (62%). However, severe forms were less recorded. Our data suggest that the changes in leptospirosis features could be related to exceptional meteorological events and their consequences on rodent populations. We propose the monitoring of rodent population and climatic data as a tool of management of leptospirosis in Guadeloupe

Portal vein thrombosis in a patient with HIV treated with a protease inhibitor-containing regimen

We report a case of an HIV seropositive female patient treated with a protease inhibitor-containing regimen who developed recurrent severe life-threathening episodes of haematemesis over time, caused by ruptured oesophageal varices as a consequence of a portal vein thrombosis. Coagulation tests revealed a protein S deficiency, an elevated homocysteinemia and a constitutional elevated plasma factor VIII coagulant activity. These coagulopathies and the HIV infection are independent risk factors for developing venous thromboembolic events. The protease inhibitor treatment may have played a role in increasing the thromboembolic risk. The recurrent bleedings only stopped after invasive surgery. The invasive splenorenal shunt operation was in this case a life-saving procedure