A retrospective evaluation of infant patients with acute lymphoblastic leukemia treated at a single institution.
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Animals↗
Biomedical subjects
G De Rossi
Publications and source records attributed to G De Rossi.
Scintigraphic assessment of swallowing after horizontal supraglottic laryngectomy.
OBJECTIVE: Oral-pharyngo-esophageal scintigraphy (OPES) proved to be able to demonstrate the pharyngeal residue of the radioactive bolus after deglutition and the presence and amount of tracheobronchial aspiration, and to calculate exactly the transit time of the various swallowing phases. The aim of this study was to evaluate the real objective effectiveness of OPES in order to assess the degree of swallowing recovery after horizontal supraglottic laryngectomy (HSL). METHODS: Nineteen patients entered the study, and 17 healthy subjects were included as control group (group 1). Nineteen patients in whom HSL had been performed at least 1 year before underwent OPES. They were divided into two groups: 13 patients (group 2) in whom the tracheal cannula had been removed and swallowing, phonatory, and respiratory functions were satisfactory and 6 patients (group 3) in whom the tracheostomy tube was still in situ for aspiration of liquids and scarring of the laryngeal vestibule. RESULTS: Our results showed that in the patients who underwent HSL, all scintigraphic semiquantitative parameters and particularly aspiration percentage values and Pharyngeal Ritention Index (PRI) at 10 and 60 seconds were able to pinpoint some residual "subclinical" alteration and/or minimal surgical sequelae frequently observed after this kind of functional surgery, even though a substantially satisfactory recovery was achieved clinically (group 2 versus group 1). In addition, aspiration amounts in group 3 were markedly higher than those in group 2 (p < .0002), and mean PRI values at 10 and 60 seconds were significantly different (p < .0001) from normal mean control values (group 1). CONCLUSION: Oral-pharyngo-esophageal scintigraphy may be regarded as a noninvasive, well-tolerated technique, with a radiation body burden within satisfactory limits of radioprotection. It appeared to be also sensitive in assessing the swallowing recovery after HSL.
Adult↗
Interstitial insertion of AF10 into the ALL1 gene in a case of infant acute lymphoblastic leukemia.
The ALL1 gene at 11q23 is a promiscuous gene participating in chromosomal abnormalities of acute leukemias with 1 of over 30 potential partner genes. Among these, the AF10 gene at band 10p12 has been recently cloned and characterized. Acute leukemias with the ALL1/AF10 chimeric gene frequently show heterogeneity in the breakpoints on 10p, as well as complex insertion (10;11) as a result of complex molecular mechanisms leading to the ALL1/AF10 fusion. In this context, we report the first description of an infant acute lymphoblastic leukemia with an interstitial insertion of the AF10 gene into the 11q23 band, resulting in the transcription of the ALL1/AF10 fusion product. Furthermore, we show how different diagnostic tools such as molecular, cytogenetic, and fluorescence in situ hybridization (FISH) analyses should be combined to resolve complex situations in the 11q23 setting.
Chromosomes, Human, Pair 10↗
Treatment of acute idiopathic thrombocytopenic purpura in children. A retrospective evaluation of 120 cases.
Acute idiopathic thrombocytopenic purpura (AITP) in children is generally a benign disease with a high frequency of spontaneous remission. Nevertheless the debate over treating or not is still open, because of the high risk of hemorrhage as long as the platelet count remains below 20 x 10(9)/l. We have retrospectively evaluated 120 pediatric cases from our center, receiving different treatments at diagnosis: no treatment (76); IVIG: 400 mg/kg/d for 5 days (28); continuous oral PDN: 1-1.5 mg/kg/d for at least two weeks (16). No patients had been previously treated for AITP. Follow-up is up to fifty months. We found no significant differences as to the percentage of responses among the three groups. We conclude that waiting without treatment is safe and appropriate in most cases; whether the hemorrhagic risk suggests treatment, standard dose continuous oral PDN and IVIG may be equally effective, but IVIG may achieve a significantly faster rise in the platelet count. The timing of treatment and the cost/benefit ratio are discussed.
Acute Disease↗
Intensive BFM chemotherapy for childhood ALL: interim analysis of the AIEOP-ALL 91 study. Associazione Italiana Ematologia Oncologia Pediatrica.
BACKGROUND AND OBJECTIVE: Since 1988 the AIEOP has used BFM-based chemotherapy for childhood ALL. Current organization and results and role of cranial irradiation in the AIEOP-ALL 91 study are reported. DESIGN AND METHODS: From 1991 to 1995, 1194 children (< 15 years) with non-B ALL, were enrolled and assigned to the standard risk [SR: age > 1 year, non-T-ALL, BFM risk factor (RF) < 0.8], intermediate risk (IR: RF > or = 0.8 but < 1.7, or with RF < 0.8 and age < 1 year, or T-ALL), or high risk [HR: RF > or = 1.7, or t(9;22), or t(4;11) or prednisone poor response or late response or CNS involvement] groups. All patients received initially protocol Ia. Thereafter SR patients received HD-MTX 2 g/m2, a modified protocol II, and continuation therapy with triple intrathecal chemotherapy (TIT); IR patients received protocol Ib, HD-MTX 5 g/m2, protocol II and continuation therapy with TIT; HR patients received 9 polychemotherapy blocks, cranial irradiation and continuation therapy. Duration of treatment was 24 months. A randomized study was conducted to evaluate the impact of high-dose asparaginase in non high risk patients: the results of this study cannot be disclosed yet. RESULTS: One thousand one hundred and fifty-two (96.5%) patients achieved CR. Overall EFS (SE) at 5-years was 71.0% (1.4), with a survival of 80.3% (1.3). Relapse occurred in 262 children (21.9%), either in the marrow (n = 192 isolated and 32 with other sites, 18.7%), in the CNS (n = 18, 1.5%), or elsewhere (n = 20, 1.7%). 5-year EFS (SE) was 83.3% (2.4) in SR, 74.7% (1.8) in IR, and 39.7% (3.5) in HR groups, respectively. INTERPRETATION AND CONCLUSIONS: Overall cure rate was higher than in the previous AIEOP-ALL 88 study. Treatment intensification with polychemotherapy blocks did not improve results in HR. Cranial irradiation can be safely omitted in over 80% of children treated with BFM based chemotherapy.
Adolescent↗
Functional and clinical relevance of CD44 variant isoform expression on B-cell chronic lymphocytic leukemia cells.
BACKGROUND AND OBJECTIVE: Recent studies have shown that expression of adhesion molecules of the Ig superfamily, of integrins and of selectins allows definition of high vs low risk B-cell chronic lymphocytic leukemia (B-CLL). The proteoglycan CD44 is an adhesion molecule that may be expressed as a standard form of 85-95 KD or as several variant isoforms. The presence of certain CD44 variant (v) isoforms on neoplastic cells indicates poor prognosis in epithelial and lymphoid malignancies, as it is associated with tumor progression and metastasis. DESIGN AND METHODS: The expression of CD44 v3, 4, 5, 6, 7, 9 and 10 was analyzed in cells from 85 B-CLL patients. Indirect immunofluorescence and flow cytometry were used to identify CD44v. Functional studies were performed by analysis of adhesion to hyaluronate (HA), one CD44 ligand, and HA-induced Ca2+ influx. A variety of statistical methods were used to define phenotypic and functional differences between the various clones, to calculate survival curves, and for multivariate analyses. RESULTS: In 17/85 B-CLL (20%), one or more CD44v were detectable by indirect immunofluorescence, whereas in 68/85 cases (80%) this technique yielded negative results. However, moAb "mixes" against CD44v and patching of surface molecules on B-CLL cells have shown that all B-CLL clones express CD44v. This has been confirmed by Western blot in a number of cases. Thus, two groups of patients whose cells bear CD44v at high or low density, are distinguished. Functions of the two clonotypes were investigated, namely their adhesion to a CD44 ligand and hyaluronate (HA), and effect on HA-induced Ca2+ influx. Cells expressing high density CD44v adhere to HA-coated substrates more efficiently than cells with low density CD44v. In all clones, HA-signaling via CD44 yields Ca2+ influx. This indicates that CD44 mediates activatory signals following interaction with the ligand. INTERPRETATION AND CONCLUSIONS: The clinical relevance of these findings has been ascertained. The 17/85 cases whose cells bore high density CD44v had significantly worse prognostic features than those of patients with low density CD44v, namely more advanced disease stage, LDT < 12 months and therapy requirement. Moreover, the median survival in the former group of patients was < 5 years as opposed to > 12 years in the latter. Therefore, analysis of CD44v expression provides indications of biological and clinical relevance also in low grade lymphoproliferative disorders.
Biomarkers, Tumor↗
Neuroimaging of cerebral and cerebellar atrophy in anorexia nervosa.
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Anorexia Nervosa↗
2'-Deoxycoformycin as treatment in refractory Langerhans cell histiocytosis.
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Enzyme Inhibitors↗
Increased serum levels of soluble CD44 standard, but not of variant isoforms v5 and v6, in B cell chronic lymphocytic leukemia.
The CD44 cell surface proteoglycan participates in a variety of functions including lymphohematopoiesis, lymphocyte homing and tumor metastasis. In addition to the standard form (CD44st), a large family of variant isoforms (CD44v) is generated by alternative splicing of a single gene. Certain CD44v (v5 and V6) are upregulated in the course of neoplastic progression and reflect the metastatic potential of tumor cells. CD44 v6 is expressed in high-grade non-Hodgkin's lymphoma cells and is released in the serum, thus providing a soluble marker that reflects tumor burden, disease progression and treatment response. Here we show that serum CD44st is elevated in approximately half of B-CLL patients. In contrast, CD44v5 and v6 are detected at normal levels in the large majority of the cases. CD44st serum levels correlate significantly with the number of circulating leukemic B cells and with the levels of another soluble B-CLL marker, beta2-microglobulin. Immunoprecipitation analyses of B-CLL sera allow detection of several high molecular weight bands and of a 78 kDa band that represents a soluble form of CD44st and is 4 kDa lower than a similar band (82 kDa) detected in B-CLL cell lysates. Elevated serum CD44st associates with a number of unfavorable prognostic factors such as high peripheral blood lymphocytosis, splenomegaly, advanced disease stage and therapy requirement. A follow-up study indicates that serum levels of CD44st are related to disease status, thus reinforcing our veiw that this molecule may represent a reliable tumor marker in B-CLL.
Adult↗
The contribution of immunoscintigraphy to the diagnosis of head and neck tumours.
Immunoscintigraphy with 111In-F(ab')2-anti-carcinoembryonic-antigen monoclonal antibody was performed in patients with primary head and neck tumours (n = 14), recurrences (n = 3) and suspected posttherapy lymph node metastases (n = 3). No false-positive but two false-negative results were obtained. Single photon emission tomography was performed 24 h after the intravenous administration of the radiopharmaceutical with a positive predictive value of 100% and a sensitivity of 90%. Only two very small (< 1 mm diameter) and necrotic primary tumours escaped detection, while the recurrences and lymph node metastases were always correctly diagnosed, in contrast to computerized tomography and ultrasound. Even though highly selective patients only were considered in the present research, the results do suggest that immunoscintigraphy may be usefully applied in all routine protocols for the study of head and neck cancer.
Aged↗
Proliferative responses to PHA, anti-CD3 and antigens in patients with lymphoproliferative disease of granular lymphocytes. Mannoproteins of Candida albicans induce proliferation and cytotoxicity.
In this paper we investigated the lymphoproliferative and cytotoxic responses of peripheral blood mononuclear cells (PBMC) of patients affected with lymphoproliferative disease of granular lymphocytes of T cell origin to mitogens and antigens. Most patients with lymphoproliferative disease of granular lymphocytes (LDGL) showed a severely impaired PBMC proliferative capacity in response to phytohemagglutinin, anti-CD3 and tetanus toxoid, an impairment that, in the case of anti-CD3 response, appears to be related to a defect of phosphorylation in response to triggering. In contrast, at least 50% of the patients had normal proliferation in response to a mannoprotein fraction purified from Candida albicans. These data suggest that some CD3+ GL from LDGL patients, that usually respond poorly to proliferative stimuli in vitro, can be triggered to perform these functions by candidal antigens.
Adult↗
Monolateral aplasia of the parotid gland.
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Adult↗
Autologous bone marrow transplantation for extramedullary relapse in childhood leukemia. The AIEOP Group and the FONOP. Italian Association of Pediatric Hemato/Oncology.
The role of autologous bone marrow transplantation (ABMT) in childhood ALL after an isolated extramedullary (IE) relapse is controversial. Between December 1984 and November 1995, 52 children underwent ABMT because of an IE relapse. The data were stored in the AIEOP-BMT Registry. Thirty four children were transplanted in 2nd CR; eighteen > 2nd CR. The median duration of 1st CR was 24 (range 3-69) and 18 (range 3-59) months, respectively. The median interval from last CR to ABMT was 6 (range 1-28) and 3 (range 1-81) months, respectively. The 5 year EFS for patients transplanted in 2nd CR was 67.7%, while the 3 year EFS for patients in > 2nd CR was 16.7%. In conclusion, ABMT was an effective treatment in early IE relapse only if performed in 2nd CR.
Adolescent↗
[An oropharyngeal-esophageal scintigraphic study of deglutition].
The aim of the present work was to evaluate whether oro-pharyngeal-esophageal scintigraphy could be used in the dynamic study of the various phases of deglutition and to determine whether it could be applied in otorhinolaryngological practice along with videofluoroscopy and videoendoscopy. The patients were divided into four different groups according to clinical features and the scintigraphic data were analyzed on this basis. Emphasis is placed on the ease of this technique, its tolerability and the low level of radiation. It is pointed out that scintigraphy is quite useful in determinating the exact percentage of the bolus inhaled into the trachealbronchial branch and in measuring the transit time of the various phases of deglutition in detail. In patients affected by upper airway-digestive tract neoplasms it is, therefore, possible to make a semiquantitative evaluation of the results of surgery. Moreover, with this method the results of any rehabilitation can be measured as well as the onset of compensation mechanisms.
Aged↗
Neuropsychological tests and [99mTc]-HM PAO SPECT in the diagnosis of Alzheimer's dementia.
Twenty-three patients with Alzheimer's dementia (AD) in relatively early stages and 40 patients with other cognitive disorders of vascular or degenerative aetiology underwent neuropsychological examination and [99mTc]-HM PAO single photon emission computed tomography (SPECT). In contrast to the commonly accepted notion of a posterior temporoparietal reduction of tracer uptake as the typical SPECT pattern of AD, the most consistent feature found in the SPECT images of our AD patients was a hippocampal uptake deficit, associated with a variable degree of temporal, parietal and frontal deficit (extending from the posterior to the anterior regions), according to the severity of the disease. These results support the theory of AD as a "hippocampal dementia", at least in the early stages. Neuropsychological tests were found to be somewhat more specific and more accurate than SPECT in distinguishing AD from non-AD cases.
Aged↗
Brain SPET and auditory cortex perfusion. Technical notes and preliminary results.
Auditory cortex stimulation was studied by 99Tcm-hexamethylpropyleneamine oxime (HMPAO) single photon emission tomography (SPET) in 11 patients with normal vestibular and auditory tests, as well as computed tomography. Markedly increased temporal and parietal blood flows were found in left brain cortex, contralateral to the stimulus. The right auditory areas showed moderate hyperactivity. The method might be useful for tonotopic mapping of auditory cortex, using various pure tonal stimuli.
Acoustic Stimulation↗
Adhesion molecule expression on B-cells from acute and chronic lymphoid leukemias.
Adhesion molecule expression on acute and chronic lymphoid leukemia cells of B lineage (B-ALL and B-CLL) may subserve several functions. Adhesion of leukemic cells to endothelial cells and to extracellular matrix components is relevant to homing, trafficking and spread of the malignant cells, and thus to clinical presentation, course and disease prognosis. Adhesive interactions between malignant cells and accessory cells, particularly stromal cells in the bone marrow environment, may support growth of the malignant cells via cytokine-delivered messages. They may also deliver signals that prevent or trigger programmed cell death of tumor cells. Here we review data on the adhesive phenotype of leukemic blasts from pro-B (CALLA +) ALL and of cells from B-CLL cases. We show that expression of certain adhesion molecules may help define disease subsets with distinctive clinical and prognostic features. One adhesion molecule, the lymphocyte homing receptor CD44, allows definition of two groups of B-CLL patients with significantly different survival.
B-Lymphocytes↗
Adhesion molecule expression on B-cell chronic lymphocytic leukemia cells: malignant cell phenotypes define distinct disease subsets.
Expression of surface adhesion molecules of the Ig superfamily (CD54 and CD58), of the integrin family (beta 1, beta 2, and beta 3 chains), of the selectin family (L-selectin), and of the lymphocyte homing receptor (CD44) was analyzed on B-cell chronic lymphocytic leukemia (B-CLL) cells from 74 patients. The aim of the study was the definition of phenotypically distinct disease subsets and the correlation of adhesion molecule phenotypes with clinical parameters. Expression of CD58 on B-CLL cells defined more advanced disease stages. In comparison with beta chain-positive cases, patients whose cells did not express beta 1, beta 2, and beta 3 integrin chains fell into the most favorable prognostic group, with lower lymphocytosis and the absence of splenomegaly, diffuse bone marrow infiltration, and therapy requirement. A novel finding was the expression of beta 3 chains on cells from a minority (12 of 74) of B-CLL cases. beta 3 chains were always coexpressed with beta 1 and beta 2 chains. Two-color immunofluorescence analyses of adhesion molecules such as alpha x beta 2 integrin (LeuM5) and L-selectin (Leu8) showed that these markers were detectable on variable proportions of leukemic cells, thus confirming the intraclonal phenotypic heterogeneity of B-CLL. Differences in the intensity of CD44 expression were also shown among the various B-CLL clones. Finally, no major variations were shown by comparison of adhesion molecule phenotypes of leukemic cells simultaneously obtained from blood and bone marrow, and of CD5+ versus CD5- clones.
Adult↗