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L Nespoli

Publications and source records attributed to L Nespoli.

103 records · Page 6Linked to original sources
PubMedDate not supplied

Recombinant human granulocyte-macrophage colony stimulating factor (rHuGM-CSF) in cyclic neutropenia.

We describe the case of a 12-year-old boy affected by cyclic neutropenia, at high risk of developing life-threatening infections, treated with recombinant human granulocyte-macrophage colony stimulating factor (rHuGM-CSF). The drug was effective in reducing the severity of neutropenia and infectious complications in our patient. It was administered for brief periods of time, in contrast to the daily continuous administration reported for rHuG-CSF. Therefore, more extensive studies must be performed to identify the most effective time schedule for the drug. In vitro studies of hemopoietic progenitor cells were useful, in this case, to predict treatment response.

Child↗
PubMedDate not supplied

[Graft vs host disease in a child undergoing allogeneic bone marrow transplantation].

GVHD has a major role in the morbidity after a BMT, even if the donors are HLA matched siblings. The risks of a severe GVHD increase when using partially matched family donors or matched unrelated donors. An acute GVHD occurs within 100 days from BMT, whereas a chronic GVHD occurs in the first year. Both acute and chronic GVHD are less frequent and severe in children. The experience of the transplant team of Pavia is in agreement with this statement. The incidence of GVHD was extremely low even then compared with the data of the Italian pediatric BMT group. In transplant performed with HLA matched donors the incidences of severe acute and chronic GVHD were 5 and 8%.

Acute Disease↗
PubMedDate not supplied

[Bone marrow transplantation in the treatment of severe aplastic anemia].

Bone marrow transplantation (BMT) in patient affected by severe aplastic anemia (SAA), is successful in 70-80% of cases, when performed with HLA identical brother or syngeneic twin as donors, and in 11-45% of cases when performed from aploidentical-identical related or HLA identical unrelated donor. The different conditioning regimens (Cy alone or in combination with TBI) have shown similar results in the long term outcomes. Cyclosporin-A is very effective in avoiding rejection and controlling GVHD.

Anemia, Aplastic↗
PubMedDate not supplied

Factors leading to the appearance, after BMT, of cytotoxic antibodies against T, B and activated T lymphocytes: critical appraisal.

In order to investigate the role of lymphocytotoxic antibodies, acquired after allogeneic and autologus bone marrow transplantation, we studied 309 sera from 42 transplanted patients (16 adults and 26 children). We tried to correlate antibody elicitation towards T, B and activated T lymphocytes with the following parameters: genetic (recipient's and donor's sex, HLA profile), clinical (recipient's primary disease, GvHD, transplant outcome) and technical (bone marrow purging, auto-or allotransplant). There is evidence that anti-T and -B cytotoxic antibodies appear earlier than anti-activated T antibodies. Anti-HLA specific antibodies seem to be produced by the transfusional stimulus: they appear early after BMT and wane after the first year. Humoral responsiveness seems to be age related (adults are more responsive than children) and conditioned by GvHD (the level of cytotoxic antibodies decreases when GvHD is prevented by bone marrow purging). The level of cytotoxicity is significantly lower in the sera of autotransplanted patients compared with the allotransplanted ones. It appears that anti-activated T antibodies are produced by cell activation at different times in adults and children: in adults this occurs during GVHD and in children during the relapse of disease.

Adolescent↗
PubMedDate not supplied

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↗
PubMedDate not supplied

[The leukemic child after therapy. Psychological aspects].

Thirty children in remission, at least one year after completing chemotherapy for Acute Lymphoblastic Leukemia, were assessed using general psychological measures. The Wechsler Intelligence tests were administered to evaluate their intellectual function: no significant reduction was found in overall IQ score. Only four of the patients, who presented neurological complications during the treatment, performed at lower levels. The group as a whole functioned poorly on tasks involving speeded performances and long-term memory. The age at diagnosis, the duration of the illness, the cranial irradiation and/or intrathecal-chemotherapy did not show any negative effects on the intellectual performance. Psycho-social adaptation was satisfactory: our patients do not show neurotic or psychotic disorders, but only affective disturbances (insecurity, irritability, poor self-perception, instability and depression). Familial adaptation was disturbed in 60% of the children and difficulty in socializing with peers was present in 57% of cases: the latter, in our experience, is related to poor self perception, present in 75% of our patients. We have found a more inadequate psycho-social adaptation in children who have not received any communication of their illness. Psychologic support can be of value for the child and his family in adapting to their difficult situation and in promoting a normal personality development of the child.

Child↗
PubMedDate not supplied

[Recent advances in histiocytosis].

"Histiocytosis" is the term currently used to describe the group of diseases characterized by activation and proliferation of monocytic-mononuclear cells. Some of these are "reactive" to well-known causes, mycobacteriae, viral and parasitic infections, or chronic storage of minerals. Wider and more intriguing is the group of histiocytosis secondary to unknown causes: sinus histiocytosis with massive lymphadenopathy; histiocytosis in the course of systemic illnesses such as rheumatoid arthritis, SLE, Crohn disease, ulcerative colity, sarcoidosis, Weber-Christian disease, Wegener granulomatosis. Histiocitytosis X is the most frequent type of histiocytosis. Hematophagocytosis is a paraphysiologic phenomenon; however, when enormously increased it is characteristic of both the virus-associated hemophagocytic syndrome and Farquhar syndrome. In some cases of severe combined immunodeficiencies (SCID) histiocytic proliferation has been observed. Finally, during the past decade the morphologic approach has led to definition of the X-linked lymphoproliferative disease (XLP) and its erroneous classification as histiocytosis. These conditions are reviewed and some clinical cases are reported.

Cell Differentiation↗