No abstract

Allogeneic hematopoietic SCT (HSCT) is currently the only curative treatment for myelodysplastic syndrome (MDS). However, many patients cannot find an HLA-matched donor. We have developed a new protocol for HLA-mismatched (including haploidentical) HSCT using G-CSF-primed BM plus G-CSF-mobilized PBSCs without in vitro T-cell depletion. A total of 36 patients diagnosed with high-risk MDS (RAEB (refractory anemia with excess blasts) or RAEBt (RAEB in transformation)) underwent transplantation from HLA-mismatched family donors. All patients achieved sustained myeloid engraftment. The cumulative incidence of grades II-IV acute GVHD (aGVHD) was 60% and that of grades III and IV aGVHD was 15%. The 2-year cumulative incidence of chronic GVHD was 56%. After a median follow-up of 17 months, 4 patients had relapsed and died and 25 patients were still alive. The 2-year probability of leukemia-free survival (LFS) was 65%. Patients transplanted within 7 months of diagnosis had better LFS (89 vs 43% ). Severe aGVHD decreased the LFS significantly by increasing non-relapse mortality (NRM). This study confirms that HLA-mismatched HSCT is a treatment option for MDS. Patients with high-risk MDS benefit from receiving HSCT early in the course of the disease.

Objective Serum soluble CD25 (sCD25) could be used as a biomarker for disease activity in conditions associated with T-cell activation including various autoimmune diseases. This study aimed to explore the role of sCD25 as an indicator of disease activity and organ involvement in patients with systemic lupus erythematosus (SLE). Methods Serum samples were collected from 107 SLE patients and 92 age-matched healthy controls (HCs). All patients were followed up for 24 weeks, and sCD25 was measured by enzyme-linked immunosorbent assay. Clinical and laboratory data were recorded at baseline and then every two weeks until week 24. The Systemic Lupus Erythematosus Disease Activity Index-2000 (SLEDAI)-2K was adopted for assessing disease activity at all visits. Results Serum sCD25 levels were significantly increased in SLE patients compared to those in HCs ( p < 0.001). More patients in the high-sCD25 group had lupus nephritis, arthritis and vasculitis ( p = 0.010, p = 0.023 and p = 0.042, respectively). SLEDAI-2K, erythrocyte sedimentation rate, C-reactive protein and 24-hour urinary protein excretion were all associated with high levels of sCD25 ( p < 0.001, p = 0.002, p = 0.038 and p = 0.029, respectively). During the 24-week follow-up, more patients in the high-sCD25 group developed renal impairment (48% vs 6.2%, p = 0.005), and higher levels of sCD25 ( p = 0.033) were found at the time of onset of renal disease. Conclusions Serum sCD25 is a hallmark of disease activity and a predictor of renal disease in patients with SLE.

Introduction To determine the presence of respiratory impairment in community-living subjects with a history of poliomyelitis. Methods In a study conducted from July 2013-January 2014, we used a national database to recruit individuals (212 males, 86 females) from north and south provinces in China with a known prior poliomyelitis infection &gt;25 years previously. 298 subjects (96.8%) completed overnight oximetry to collect the number/hr of drops in oxygen saturation &gt;4% (ODI4) and Epworth Sleepiness Scale (ESS); many completed a metabolic and lipid panel, arterial blood gas analysis, chest x-ray (CXR), spirometry and maximal voluntary ventilation (MVV). Some completed risk profiling for OSA. Results Age was 47.8 ± 6.7 years (M±SD) and, when known, the age of infection was 2.3 ± 1.8 yrs. As defined by ODI4 ≥ 5/hr, the frequency of sleep-disordered breathing (SDB) was 37.2%; 9% (n = 27) had an ODI4 ≥15/hr. Those with vs. those without SDB differed by male gender (81% vs 65%, p = 0.004) and BMI (25.9 vs 23.0kg/m2, p &lt; 0.001). ESS was within the normal range, but was higher in those with 6.8 ± 5.0 vs. without 5.2 ± 4.0 (p &lt; 0.01) SDB. Spirometry and MVV revealed no differences among groups. Scoliosis on the CXR was present 26.1% of those with and 14.4% of those without SDB. ODI4 correlated weakly with body mass index (r = 0.40). Glucose levels or hyperglycemia were not different. A triglyceride level &gt; 1.7 mmol/L was present in 47.2% of those with and 21.3% of those without SDB (p &lt; 0.001). Conclusion Mild (28%) and to a smaller extent moderate-severe (9%) SDB is present many years after surviving poliomyelitis in infancy. In most, sleepiness is low but scoliosis is often present, and associations to hyperlipidemia and obesity are not reflected in fasting glucose levels. Support no

No abstract

We are the first to demonstrate the reliability of the SIL-TAL1 fusion gene as a good MRD marker for patients with T-ALL after allo-HSCT.

Results: 15 patients were included: 11 women and 4 men, with a mean age of 42.2 years (SD+/-16) The genetic study was performed in 12 patients: 75% were positive for HLA DQ2 and 25% for HLA DQ8. The determination of antibodies was positive in 12 patients (73%) and negative in 3 (27%). These 3 were positive for the genetic study (2 HLA DQ2 and 1 HLA DQ8). The study of BD showed: type I in 4 patients; Type IIIa in 2 patients; Type IIIb in 6 patients; Type IIIC in 3 patients. BMD showed normal values in 33%, osteopenia in 47% and osteoporosis (OP) in 20% of patients. Thus, 67% had an alteration in BMD at the time of diagnosis. 100% had a Vit D deficit, with a mean value of 14.3 ng/mL (range 4.15-26.8). 75% of them had values below 20 ng/mL (40% less than 10 ng/mL). 40% had secondary hyperparathyroidism (SHP), 83% of which had an alteration in BMD (67% osteopenia and 16% OP). The BD of the patients with SHP and alteration of the BMD showed a histological pattern type Marsh III. No patient presented an alteration in the values of calcemia, phosphataemia or calciuria. The relationship between the degree of alteration in BMD and the Marsh classification (table) was studied. 72% of patients with a Marsh III had an alteration in BMD (3 OP and 5 osteopenia vs 3 normal).