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A Lanfranchi

Publications and source records attributed to A Lanfranchi.

62 records · Page 4Linked to original sources

Functional depletion of T cells by vincristine and methylprednisolone as an in vitro model for the prevention of graft versus host disease.

BACKGROUND AND METHODS. The outcome of mismatched bone marrow transplantation is still severely hampered by graft versus host disease (GVHD) and graft rejection. Procedures for the recognition and selective elimination of T cells are still unsatisfactory due to the increased incidence of graft failure and late rejection. Lymphocyte proliferation and generation of cytotoxic T cells (CTL) in response to allogeneic cells are considered good in vitro correlates of GVHD and have been used in the present study to asses the capacity of two drugs (vincristine, VCR, and methylprednisolone, MP) to affect the T cells involved in these reactions. RESULTS AND CONCLUSION. Treatment in vitro with VCR and MP has been shown to inhibit the functional capacity of peripheral blood lymphocytes to proliferate (mean reduction 95.8%) and to generate CTL in response to haploidentical stimulator cells. Responsiveness to antigens and mitogens was affected to a minor extent (mean reduction 40% and 65.5%, respectively), and the method allowed recovery of hemopoietic precursors. The results suggest that treatment of donor bone marrow with VCR and MP is worth studying as a new approach to the prevention of GVHD in haploidentical bone marrow transplantation.

Bone Marrow Cells↗

[Cellular and molecular therapy in severe combined immunodeficiencies].

Severe combined immunodeficiencies are usually fatal diseases unless affected children are admitted to protective isolation unit or unless the underlying immunological defect is treated by transplanting bone marrow from an healthy donor. The patients present, with early onset, life-threatening infections from fungal, viral or bacterial agents. Since only a minority of patients has an HLA-identical donor, recently other strategies have been devised including bone marrow transplantation from donors other than HLA-identical within the family or from HLA-non identical family members or from HLA-matched unrelated donors included in the International Bone Marrow Volunteers' Registry. In these cases, in order to realize this approach the "purging" of T-cells of the HLA-non identical donor bone marrow is necessary. Overall survival after HLA-identical BMT is 76%, when all BMT of the European multicenter analysis are considered, while in BMT from non-identical donors is 56%. Recently particular cases of SCID caused by enzyme deficiency, such as adenosine-deaminase (ADA), have been treated by molecular therapy with administration of polyethylene glycol (PEG) conjugated ADA: PEG protects from degradation and inhibits clearance of the enzyme. This approach, already realized in 15 children, allows reconstitution of cellular and humoral immunity, as demonstrated by one case treated by our group.

Adenosine Deaminase↗

[Bone marrow transplantation in congenital defects of immunity].

BMT can cure several congenital immunological defects: if in these disease the engrafting is easier, the GVH reactions are more frequent and severe. The possibility to deplete from T lymphocyte the marrow before infusion, has overcame this difficulty. From 1968 183 BMT have been performed in Europe on patients with SCID (70 from HLA-identical donor, 113 from HLA-nonidentical donor). The survival after 2 years is 76% in the first group, and 56% in the second group (100 marrows have been T-depleted with different techniques). Strict isolation procedures before the transplant are very important to achieve good results. The possibility to treat different immunodeficiency With BMT are also discussed.

Bone Marrow Transplantation↗

Successful lectin-separated bone marrow transplantation in adenosine deaminase deficiency-related severe immunodeficiency.

Severe combined immunodeficiency disease (SCID) with adenosine deaminase (ADA) deficiency is a genetic autosomic recessive disorder with profound impairment of T-cell function, invariably complicated by fatal infections. The absence of ADA-enzyme and the accumulation of deoxy-ATP, with toxic effects on the T-lymphocytes is the common feature of this disease. As a consequence, lymphoid precursors failure to develop into mature T-cells, resulting in absolute lymphopenia and atrophy of the thymus. Bone marrow transplantation from an HLA-identical donor is considered the treatment of choice for this disease. We describe the case of a 1-month-old child with ADA deficiency SCID who underwent bone marrow transplantation (BMT) using paternal haploidentical, lectin-separated marrow, as a source of hemopoietic stem cells.

Adenosine Deaminase↗

Monoclonal expansion of large granular lymphocytes with a CD4+ CD8dim+/- phenotype associated with hairy cell leukemia.

Peripheral blood lymphoid cell expansions with an unusual CD3+, CD4+, CD8dim+/-, CD11b+, CD57+ immunophenotype have recently been reported. They frequently have the morphology of large granular lymphocytes (LGL) and can be either monoclonal or polyclonal. Their significance is still unclear and no association with hematological neoplasms has been described. We report the case of a patient with a monoclonal expansion of LGL associated with a B-cell-derived hairy cell leukemia. The two lymphoid clones were not physically associated since T-LGL were found in the peripheral blood and hairy cells were detected in the bone marrow and kidney.

Aged↗

["...at least in my village, it is the custom...". From stagnation to transformation of familial reality constructs in immigrants].

The overrepresentation of immigrant children in special-education facilities and their underrepresentation in the stationary and ambulatory child and adolescent psychiatric services is becoming more and more alarming. It is inadequate to understand the reality of immigrant families by focusing on the concept of the "back and forth" between two cultures. It is more adequate to understand the reality of immigrant families by making use of the concept of the "construct of transitional realities". This newer and more relevant concept of understanding the reality or situation of immigrant families is based on the method of ethnobiographic case reconstruction and case comparison. Through this new approach, it is possible to discern the ways and means by which some families living in a foreign culture achieve a more or less autonomous mode of living and how they succeed in overcoming difficulties when going from one frame of reference to another or from one phase of life to another. This report presents excerpts from a study of southern Italian families living in Switzerland. As drawn from the collected material, these families can be typified as: traditional-progressive, traditional-sclerotic, traditional-regressive. In analyzing these individual family biographies and the various constructs of problem solving, the general societal background is always taken into consideration by the author. Therefore, the three types of families are neither singular nor particular but rather universal.

Acculturation↗