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C Rosanda

Publications and source records attributed to C Rosanda.

30 records · Page 2Linked to original sources

Massively diffuse multifocal granulocytic sarcoma in a child with acute myeloid leukemia.

A case of granulocytic sarcoma in an 8-year-old boy with acute myeloid leukemia and t (8; 21) is reported. The case is of interest due to massive extension of the tumor, which may raise different diagnostic difficulties with other solid tumors such as lymphoma, Ewing sarcoma, and soft tissue sarcoma. Furthermore, the tumor was localized in some sites, such as the parotid region and peripheral nerves, which are not usually involved in granulocytic sarcoma. The case points out the diagnostic difficulties with this kind of tumor and appears to contribute to the identification of a subgroup of acute myeloid leukemia with peculiar features, such as M2 morphology with Auer rods, t (8; 21), granulocytic sarcoma and a poor prognosis.

Child↗

Granular acute lymphoblastic leukemia in children. "Aieop Cooperative Group for Cytology of Acute Leukemias".

BACKGROUND: Granular acute lymphoblastic leukemia (ALL) is a rare morphological variant of ALL, characterized by cytoplasmic azurophil granules or inclusions, positive for aspecific esterase and acid phosphatase, with heterogeneous features at the ultrastructural level. METHODS: In an attempt to determine whether the presence of granules or inclusions marks a biologically distinct variety of ALL with peculiar clinical features, a prospective morphological review was undertaken of children entering AIEOP protocols for ALL in the period from 1985 to 1989. RESULTS: Of 531 cases examined, 16 (3%) were found to have greater than 1% granular bone marrow blasts, with 7 cases (1.3%) having greater than 10%. The presence of granules or inclusions was associated with the immunophenotype of "common" ALL. There was no clear association with FAB type L1 or L2 nor with particular clinical or hematological findings at presentation. Complete remission was achieved in all cases; one patient died of infection in remission at 3 months and 2 patients relapsed after 12 and 32 months, respectively, while the others are still in remission after a minimum follow-up of 24 months. CONCLUSIONS: In conclusion, granular morphology seems to have no prognostic importance in children ALL.

Acid Phosphatase↗

B-cell acute lymphoblastic leukemia (B-ALL): a report of 17 pediatric cases.

BACKGROUND AND METHODS: B-cell acute lymphoblastic leukemia (B-ALL) presents FAB L3 morphology and surface Ig, CD19, CD20, CD24. This pattern may show some morphological and immunological heterogeneity. Seventeen pediatric cases of B-ALL at onset, treated in twelve AIEOP Centers (Italian Association for Pediatric Hematology and Oncology), are presented. RESULTS AND CONCLUSIONS: Clinical and hematological features were characterized by low WBC counts at presentation, high M/F ratio, older age and association with extramedullary involvement. The overall survival curve at 78 months is 40%. All patients showed blasts positive for surface Ig (sIg), DR, CD19, and CD24. Ten/17 cases presented the classical features of B-ALL: FAB L3 morphology, sIg+ restricted to light chains, CD20+, cytoplasm mu (c mu)-, CD10-, TdT-. The remainder showed some differences in this pattern, such as non-L3 morphology (3 cases), absence of CD20 (3 cases), CD10+ (4 cases), TdT+ (3 cases), c mu+ (1 case), lack of surface light chains (1 case). This rare ALL subset seems to be characterized by a high phenotype heterogeneity, indicating various degrees of differentiation.

Adolescent↗

Cytogenetics of infantile leukemias and its correlations with bio-clinical features. The "G. Gaslini" Children's Hospital experience over a 9-year period.

BACKGROUND AND METHODS: Infantile leukemia is a rare disorder, and few cytogenetic studies have been performed on this condition. RESULTS AND CONCLUSIONS: The authors present the cytogenetic analyses performed on 14 cases of infantile leukemia. The most frequent chromosomal changes are rearrangements involving 11q (4 cases) and gains of one or more chromosomes 21. Patients with chromosomal rearrangements show a worse prognosis than those with only hyperdiploidy or a normal karyotype, although the difference was not statistically significant due to the small size and short median follow-up.

Chromosome Aberrations↗

New clinical aspects of Pearson's syndrome. Report of three cases.

We describe 3 patients affected by Pearson's syndrome, presenting anemia, exocrine pancreas failure, and skeletal abnormalities; insulin-dependent diabetes mellitus arose in two cases during the course of the disease. Bone marrow dysplasia and exocrine pancreas failure are also reported in Shwachman's syndrome; the two forms differ in bone marrow morphology. The clinical pattern of Pearson's syndrome can be so polymorphic as to increase the difficulties of differential diagnosis with Shwachman's syndrome.

Bone Marrow↗

A short-term in vitro drug sensitivity assay in pediatric malignancies.

A short-term in vitro chemosensitivity test has been applied to 131 specimens from 109 children with various malignancies. The clinical outcome was correlated with in vitro percentage inhibition (PI) of DNA synthesis by the drug tested. In 62 correlations, PIs of responsive and of resistant patients differed significantly, with a cut-off value of 37%. The test predicted clinical resistance in 53/54 cases, with a specificity of 98%, and clinical response in 8/8 cases, with a sensitivity of 100%. This test can be useful particularly in predicting chemoresistance in relapsing patients, to avoid unnecessary toxicity.

Child↗

[Neuroblastoma at the Giannina Gaslini Institute. Analysis of 94 cases diagnosed 1972-1981].

The authors have analysed 94 consecutive previously untreated children affected by NB diagnosed at the Giannina Gaslini Children's Hospital in the period January 1972 - December 1981. Age at diagnosis ranged between 1 month - 16 years (median 2 years). Diagnosis was made on histological grounds in 82 cases, on clinical, instrumental and laboratory data in the remaining 12 cases. Evans' staging system was adopted for classifying the disease extent. Patient recruitment was 9.4 cases for year. Female sex slightly prevailed in our series. 17 patients had localized disease (stage I and II), all surviving since 27 - 90 months. 28 children had regional disease (stage III) half of whom are presently alive. Five of the 47 cases with disseminated disease are alive at the time of this study; only one of these 5 can be considered cured, having been followed since more than 4 years. One of the two IV-s stage children survives disease-free at 89 months. 49% of patients were 2-year old or less at diagnosis while only 2 patients were older than 10. Survival was best in children diagnosed under one year of age, lowest in the 2 - 6 year age group. 74% of patients had their primary located in the abdomen (36 in the adrenal, 16 in sympathetic ganglia, 18 in un unidentifable site). Prognosis was worse in these patients compared with those with primary in the thorax, neck and pelvis. The clinical presentation of the disease has been extremely various: most patients had a diagnosis different from the definitive one, and were accordingly treated usually for several weeks or even months. Among laboratory data, urinary catecholamine metabolites, serum LDH and serum ferritin represented the most suitable indices of disease activity. Among immunological studies, none has shown a good correlation with the extent of the disease, nor with the clinical course. Stage I children had only surgery as treatment, while in stage II surgical ablation was followed by short-lasting chemotherapy and radiotherapy in few cases. In stage III and IV more complex multidisciplinary approach was utilized in the attempt to achieve a complete tumor regression. While about a half stage III cases appears curable by these treatment modalities, little impact has been demonstrated by this therapy in widespread disease, although the use of well-designed protocols seems have improved the complete remission rate and the median length of survival.

Adolescent↗

Evaluation of minimal residual disease in high risk childhood acute lymphoblastic leukemia using an immunological approach during complete remission. AIEOP Cooperative Group for Immunology of Acute Leukemias.

BACKGROUND: Sensitive methods for detecting residual disease may complement conventional morphology while monitoring the response to treatment in leukemia patients. METHODS: We studied minimal residual disease (MRD) by selecting via a colony assay a peripheral blood (PB) cell population enriched in putative malignant cells and detecting occult leukemic cells by double immunologic analysis performed on colonyforming cells (CFC). Using this combined technique we assayed the PB of high risk children with acute lymphoblastic leukemia (ALL) in order to demonstrate possible differences in "the residual tumor cell burden" among leukemia patients and to correlate these with a 3-year clinical follow-up. RESULTS: In all 22 patients positive results were obtained for up to 18 months following induction chemotherapy at times of apparent hematologic remission. Common ALL (cALL) patients exhibited a mean of 9.6% cAlla+ cells (range: 2% to 30%), whereas cALL antigen (cALLA) and cALLA/Tdt or CD1a/Tdt combination were never found in the colonies derived from healthy individuals. Six out of 22 cALL patients expressed a mean of 16.5% cALLA+/Tdt+ CFC (range: 2% to 35%). Five T-ALL children presented a mean of 24% CD1a/Tdt+ cells (range: 8% to 44%). Extensive follow-up indicates a correlation between the percentage of CFC Tdt+/lymphoid marker+ cells (more than 10%) and subsequent clinical relapse. In one patient relapse occurred after 16 months with a dramatic increase in the number of leukemic cells. In contrast, the decline in malignant cells, observed in two cases, predicted a favourable course. Five patients tested before autologous bone marrow transplantation (ABMT) presented high number of positive cells and relapsed at various times. CONCLUSIONS: We conclude that this approach to the study of MRD could be valuable in monitoring the efficacy of chemotherapy, as well in evaluating the quality of purged marrow.

Adolescent↗

[The morphological assessment of bone marrow infiltration in neuroblastoma].

Bone marrow biopsy is very important in diagnosis and follow-up of children affected by neuroblastoma (NB). Between June 1995 and May 1997 we studied 55 patients with NB stage 4. Specimens were obtained at the diagnosis (in 8 patients) and after chemotherapy (in 55 patients) in order to evaluate the effects of treatment on bone marrow disease. 88% of 343 biopsies were representative versus 99% of 639 aspirates. Of 8 stage 4 patients evaluated at diagnosis, 15/16 biopsies versus 9/15 aspirates were positive. Following chemotherapy, out of 298 evaluable sites examined, 111 biopsies versus 30 aspirates (37 versus 10%) were positive. Of 111 positive biopsies 53 showed a focal pattern (35 differentiated, 18 undifferentiated), while 51 showed a diffuse pattern (18 differentiated, 40 undifferentiated). Our results confirm previous literature data indicating a better efficacy of histology versus morphology in detecting residual bone marrow disease (especially in case of focal differentiated pattern). The recent introduction of a specific monoclonal antibody, called anti-GD2, has improved our capacity to detect minimal residual disease in patients' bone marrow. The inclusion of anti-GD2 immunohistochemistry in our evaluation will possibly increase our overall sensitivity to detect minimal residual disease and may provide information capable to direct the physician's decision into a more rational patient's treatment.

Adolescent↗

[The identification of minimal residual disease in the bone marrow and peripheral blood in neuroblastoma. The prognostic and therapeutic implications].

A highly sensitive and specific methodology to detect neuroblastoma cells in the bone marrow and peripheral blood of children with neuroblastoma is of critical importance for proper staging and treatment of these patients. In addition, patients with bone marrow infiltration at diagnosis need to undergo regular investigation to measure the effectiveness of chemotherapy (so called "in vivo" purging). Finally, the evaluation of autologous stem cells taken from bone marrow or peripheral blood is necessary to rule out or minimise the possibility of reinfusing tumor cells to the patient following myeloablative therapy. The authors provide a "state of the art" data on this complicated issue and give their preliminary results of their own experience, mainly concerning the immunocytological methods.

Biomarkers, Tumor↗