Publications by authors named "Federica Bolda"
Background: Minimal residual disease (MRD) monitoring is an important tool to optimally address post-transplant management of acute myeloid leukemia (AML) patients.
Methods: We retrospectively analyzed the impact of bone marrow CD34+ molecular chimerism and on the outcome of a consecutive series of 168 AML patients submitted to allogeneic stem cell transplantation.
Results: The cumulative incidence of relapse (CIR) was significantly lower in patients with donor chimerism on CD34+ cells ≥ 97.
Less than 25% of children who require hematopoietic stem cell transplantation (HSCT) for primary immunodeficiencies (PIDs) or genetic hematological diseases have an HLA-identical sibling. For them, a matched unrelated donor (MUD), although baring a greater risk of graft failure, delayed engraftment and immune reconstitution, and severe graft-versus-host disease (GvHD), represents a valid alternative. The stem cell source is also important, as unprocessed peripheral blood stem cells (PBSCs) contain 5 to 10 times more T cells than bone marrow (BM)-derived grafts, a major risk especially for small children with PID.
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- A child with severe combined immunodeficiency (SCID) was found to have a rare case of maternal T-cell engraftment alongside adenosine deaminase (ADA) deficiency, a situation not previously documented.
- Initial diagnosis suggested conventional SCID due to misleading presence of maternal T cells and a lack of classic ADA deficiency symptoms, but further testing confirmed ADA-SCID.
- Treatment included polyethylene glycol-modified bovine ADA therapy, which reduced maternal T cells, followed by successful bone marrow transplantation from an unrelated donor, leading to complete immune system recovery.
Purpose: We carried out a retrospective analysis of 27 patients with Adenosine Deaminase (ADA) deficiency diagnosed in a single center from 1997 to the 2013, for evaluating whether data regarding types of disease-inducing mutations, biochemical and immunological features as well as clinical outcomes of patients treated with enzyme replacement or transplantation, were comparable to those obtained in multicenter studies.
Methods: The ADA deficiency diagnosis was performed with biochemical, immunological and molecular techniques. Ten patients treated with hematopoietic stem cell transplantation and three in treatment with enzyme replacement were followed up in our center.
Article Synopsis
- Imatinib mesylate (IM) is the main treatment for Chronic Myeloid Leukemia (CML) and is necessary for long-term survival, but stopping it may lead to relapse.
- Traditional tests for checking minimal residual disease are not very sensitive, but a new genomic DNA Q-PCR assay has been developed to more accurately detect the presence of leukemic cells.
- In a study of 8 CML patients on IM for over 8 years, the new gDNA Q-PCR method revealed persistent leukemic cells in nearly one-third of cases where traditional mRNA tests showed undetectable levels, suggesting it could improve decision-making for treatment.
Levels of Kappa-deleting recombination excision circles (KRECs), T-cell receptor excision circles (TRECs), and T-cell repertoire diversity were evaluated in 1038 samples of 124 children with primary immunodeficiency, of whom 102 (54 with severe combined immunodeficiency and 48 with other types of immunodeficiency) underwent hematopoietic stem cell transplantation. Twenty-two not transplanted patients with primary immunodeficiency were used as controls. Only data of patients from whom at least five samples were sent to the clinical laboratory for routine monitoring of lymphocyte reconstitutions were included in the analysis.
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