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Biomedical subjects

F Mandelli

Publications and source records attributed to F Mandelli.

At least 451 records · Page 25Linked to original sources

Acute non lymphoid leukaemia following Hodgkin's disease. Clinical, biological and cytogenetic aspects of 3 cases.

In a groups of 254 patients treated for Hodgkin's disease with a follow up period of minimum 2 years, 3 cases of acute non lymphoid leukaemia (ANLL) were observed: erythroleukaemia, myelomonocytic and myeloblastic leukaemia, respectively. The crude incidence of leukaemia in all patients was 0.0128 and patient year risk was estimated to be 0.003652. All 3 patients had received radiation therapy and chemotherapy. In all cases of haemopoietic dysplasia preceded ANLL. Bone marrow chromosome investigations showed an abnormal karyotype in all patients: chromosomal changes were present in 100% of cells and revealed a non-random distribution, the most frequent involvement being clustered to chromosomes nos 11, 17 and 21. Hypodiploidy was prevalent and multiple structural rearrangements, such as markers, rings and minutes, were present in a high percentage of cells. Other changes involved chromosomes nos 5, 7 and 14. Our results are compared with other previously reported cases and possible pathogenetic implications are discussed.

Acute Disease↗

Y chromosome duplication: a minor route evolutive pattern in CML.

2 patients have been investigated, in which Y chromosome duplication occurred during blastic transformation of chronic myelocytic leukaemia. Comparison of cytogenetic findings and survival data in our cases and in previously reported individuals, suggests preliminary conclusions about the prognostic significance of this aneuploidy. Y chromosome gain does not seem to represent per se an unfavourable event, unless it is associated with additional chromosome change.

Aneuploidy↗

Lack of prognostic significance of the pretreatment labeling and mitotic indices of marrow blasts in acute nonlymphocytic leukemia (ANLL).

The prognostic significance of the pretreatment growth characteristics of marrow blasts was examined in 37 patients with acute nonlymphocytic leukemia (ANLL) treated with a similar therapeutic regimen. Initial mitotic index (MI) and in vitro 3H-thymidine labeling index (LI) were broadly distributed showing no correlation to other initial variables, such as age or absolute blast count. No relationship whatsoever was observed between the pretreatment LI, MI and either the likelihood of achieving a complete remission or the remission and survival length. We conclude that the initial growth characteristics of marrow blasts do not play a significant role in predicting the therapeutic response in ANLL; age, modality of therapy and, possibly, the dynamic perturbations of the proliferative activity of the blast cells induced by treatment should be regarded as more reliable prognostic indicators in ANLL.

Acute Disease↗

Daunomycin, cytosine arabinoside and 6-thioguanine (DAT) vs vincristine, cytosine arabinoside and 6-thioguanine (VAT) in the induction treatment of acute nonlymphocyte leukemia: a randomized collaborative study.

One hundred patients were entered in a cooperative study comparing the efficacy of two different regimens in the induction treatment of acute nonlymphocytic leukemia (ANLL). Patients were randomly allocated to receive either the DAT or VAT combination; half of the patients were also randomized to receive CNS prophylaxis including intrathecal methotrexate + prednisone and cranial irradiation. Consolidation and maintenance therapy were uniform in responding patients. Out of 82 evaluable patients 41 (50%) attained complete remission (CR) with no significant difference between the two regimens. Median remission duration was slightly longer in the DAT group (32.5 vs 22 weeks); median survival was 34 weeks for all evaluable patients with no difference between the two schedules. Meningeal relapse occurred only in two patients after 19 and 99 weeks of continuous remission. Fourteen patients are still alive after 61 to greater than or equal to 155 weeks, of whom seven are in their initial remission (six in the DAT and one in the VAT group). We conclude that 1) DAT and VAT are equally effective in inducing CR in a high proportion of ANLL patients; 2) until marrow remission can be prolonged significantly, preventing CNS leukemia will not have any significant impact of the course of ANLL.

Adolescent↗

Immunological rebound following cessation of chemotherapy in 15 acute lymphoid leukemia patients in complete remission.

The number of blood and bone marrow lymphocytes, the E-rosette forming cells, the Ig-bearing lymphocytes, the serum immunoglobulin levels were evaluated after discontinuation of 36 months of chemotherapy in 15 acute lymphocytic leukemia patients in complete remission. The study provides further information on the immunological rebound during the "off-therapy" period.

Antineoplastic Agents↗

Preliminary results of the combination methotrexate-asparaginase in patients with acute non lymphoid leukemia.

Twenty-three refractory and/or relapsing acute non-lymphoid leukemia patients were treated with a combination of methotrexate (MTX) and asparaginase (Aase) slightly modifed from that recently proposed by Capizzi et al. Patients received MTX on day 1, followed by Aase 24 hours later; the two day course was repeated weekly. The starting dose of MTX was 60 mg/m2 and was increased by 50% at each course to maximum toxicity. The dose of Aase was kept constant at 10,000 u/m2. Twelve patients achieved complete remission lasting 3 to 58 weeks, with 8 patients surviving more than 30 weeks. Toxicity has been remarkably mild with the major problem being hypersensitivity to Aase.

Adolescent↗

Solitary secretory plasmacytoma of the lung in a 14-year-old boy.

A case of solitary plasmacytoma of the lung in a 14-year-old boy is reported. The diagnosis was made after a lobectomy was performed for the presence of a mass in the right lung. Two aspects of this case are of particular interest: the young age of the patient and the disappearance after 2 months of the M-protein of of the IgG-k type which was present in the serum.

Adolescent↗

A CRM-Positive variant of factor-VII deficiency and the detection of heterozygotes with the assay of factor-like antigen.

Nine patients with severe factor-VII deficiency, belonging to seven pedigrees were studied for the presence of factor-VII-CRM with an inhibitor neutralization assay. The antibody, raised in rabbits, did not precipitate the antigen and could only be used in a fluid phase assay to measure the capacity of plasma to neutralize inhibitory activity directed against factor-VII activity. In one of these nine patients normal amounts of factor-VII-CRM could be demonstrated. The CRM + patient did not show a clinical picture at variance with that of the CRM-patients. The investigation into this CRM+ pedigree revealed heterozygosity in nine out of 12 persons when using the ratio between biological factor-VII activity and factor-VII-CRM as the criterion.

Adolescent↗

Experience on the treatment of chronic myelocytic leukaemia (CML) in blastic crisis.

A group of 50 patients with chronic myelocytic leukaemia in blastic crisis were treated with different chemotherapeutic regimens. A total of 19 remissions (6 CR + 13 PR) were achieved with an overall and complete response rate of 38% and 12% respectively. Of the 4 patients presenting with a lymphoblast-like appearance of the blasts, the 3 treated with the combination of vincristine and prednisone all achieved remission (2 CR + 1 PR). Duration of survival was significantly longer in responding patients (median 11.5 versus 4.5 months). Either more effective therapeutic regimens or prevention by aggressively approaching the chronic phase are to be sought in order to improve the prognosis of blastic crisis of CML.

Adolescent↗

Glucose-6-phosphate dehydrogenase Velletri.

A new variant of red cell glucose-6-phosphate dehydrogenase (G6PD) has been found in a Caucasian man with congenital non-spherocytic haemolytic anaemia. This variant has reduced activity, increased thermolability, increased Michaelis constants for glucose-6-phosphate and NADP, slightly increased electrophoretic mobility, and a biphasic pH-activity profile. The red cell adenine compounds and ATP, are in normal limits. The increased activity of red cell NADP-glutathione reductase is probably the expression of a mechanism of compensation for the decrease of G6PD and a consequence of the decrease of NADPH.

Anemia, Hemolytic, Congenital Nonspherocytic↗