The Latin American experience of allografting patients with severe aplastic anaemia: real-world data on the impact of stem cell source and ATG administration in HLA-identical sibling transplants.

We studied 298 patients with severe aplastic anaemia (SAA) allografted in four Latin American countries. The source of cells was bone marrow (BM) in 94 patients and PBSCs in 204 patients. Engraftment failed in 8.1% of recipients with no difference between BM and PBSCs (P=0.08). Incidence of acute GvHD (aGvHD) for BM and PBSCs was 30% vs 32% (P=0.18), and for grades III-IV was 2.6% vs 11.6% (P=0.01). Chronic GvHD (cGvHD) between BM and PBSCs was 37% vs 59% (P=0.002) and extensive 5% vs 23.6% (P=0.01). OS was 74% vs 76% for BM vs PBSCs (P=0.95). Event-free survival was superior in patients conditioned with anti-thymocyte globulin (ATG)-based regimens compared with other regimens (79% vs 61%, P=0.001) as excessive secondary graft failure was seen with other regimens (10% vs 26%, P=0.005) respectively. In multivariate analysis, aGvHD II-IV (hazard ratio (HR) 2.50, confidence interval (CI) 1.1-5.6, P=0.02) and aGvHD III-IV (HR 8.3 CI 3.4-20.2, P<0.001) proved to be independent negative predictors of survival. In conclusion, BM as a source of cells and ATG-based regimens should be standard because of higher GvHD incidence with PBSCs, although the latter combining with ATG in the conditioning regimen could be an option in selected high-risk patients.

Idiopathic dilated cardiomyopathy. Elastic parallel element dysfunction as a physiopathological hypothesis for ventricular failure.

BACKGROUND: Idiopathic dilated cardiomyopathy is a disease of unknown etiology, although the viral-immunologic pathogenesis has recently emerged as an important hypothesis. Its distinctive anatomopathologic features are: macroscopically, a great ventricular dilation with little hypertrophy, and microscopically marked diffuse interstitial fibrosis not observed in other pathologic entities with dilation. Hemodynamically, its main characteristic is a progressive loss of the systolic function, although the diastolic function is also impaired. To date it is accepted that in dilated states ventricular remodeling occurs due to sliding of fiber with a maximal sarcomere distention; it is also assumed that the ventricular dysfunction is due to a primary deficit in contractility caused by the injury and loss of myocites. HYPOTHESIS: The aggressive agent mainly attacks the interstitial tissue, thus damaging the elastic parallel element structures. This results in a loss of absorbing power during diastole, starting a progressive dilation which results in maximum sarcomere distention, and compromises the ventricular function. The organ response is to create a new parallel element, which results in an increased fibrosis which also compromises this function.