PubMed Health⌕ Search

Biomedical subjects

R Giardini

Publications and source records attributed to R Giardini.

82 records · Page 5Linked to original sources

Disseminated Burkitt's lymphoma after kidney transplantation: a case report in a boy with Drash syndrome.

PURPOSE: We discuss the clinical, laboratory findings and treatment of a boy who developed Burkitt's lymphoma (BL) after renal transplant and some issues about lymphoproliferative disorders after transplantation. METHODS: A 6-year-old boy with Drash syndrome developed disseminated Burkitt's lymphoma 38 months after transplantation for renal failure. Immunosuppressive therapy had consisted of prednisolone and cyclosporine. Polychemotherapy was initiated. RESULTS: Polychemotherapy induced rapid and complete remission of the disease without major side effects despite the renal transplant allograft and prolonged immunosuppression. CONCLUSIONS: A child with posttransplantation B-cell high-grade lymphoma can be successfully treated with the same chemotherapy regimen used for ordinary cases.

Antineoplastic Combined Chemotherapy Protocols↗

Central nervous system involvement in non-Hodgkin's lymphomas: value of lumbar puncture as initial staging procedure.

To identify the subgroups of patients with malignant non-Hodgkin's lymphomas who might benefit from prophylactic therapy to prevent CNS relapse, lumbar puncture was routinely performed among the other staging procedures from January 1976 to October 1979 in 76 patients with diffuse lymphomas. The study also takes into consideration 32 patients who came to out observation during the same period and who were studied with lumbar puncture performed in case of suspicious CNS involvement or along with other procedures during restaging the acquire further information on prognostic factors related to CNS involvement. Cerebrospinal fluid (CSF) cytology was positive in 3 of 76 patients studied with initial lumbar puncture; however, only 2 (2.6%) were asymptomatic. Within the group of 32 patients in whom lumbar puncture was performed during the course of the disease, all 17 patients with suspicious CNS involvement were found to have a positive CSF cytology with the exception of 1 patients with multiple focal involvement of brain parenchyma. Clinical signs of CNS involvement associated with CNS positivity were all found in patients with diffuse histology. In these patients bone marrow invasion or a leukemic picture was frequently associated with CNS relapse. In 47% of patients CNS disease developed while they were in clinical remission. In this series cranio-spinal irradiation associated with intrathecal chemotherapy provided the best results, even in survival was not primarily dependent upon the control of their CNS involvement but related to progressive systemic disease in other sites. On the basis of the clinico-prognostic parameters examined, some guidelines for early diagnosis and treatment of CNS lymphomatous involvement are provided.

Bone Marrow↗

Retrospective analysis of ploidy in primary osseous and extraosseous Ewing family tumors in children.

AIMS: To retrospectively study the DNA content in a series of childhood Ewing Family Tumors (EFT), and to investigate its prognostic value. METHODS: The study was performed on a series of 27 EFTs (osseous Ewing's sarcoma, 18 cases; extraosseous Ewing's sarcoma, 2; peripheral neuroepithelioma, 4; Askin Rosai tumors, 3). Ploidy was investigated using both flow cytometry (FCM) and image cytometry (ICM) on tumor cell suspensions from formalin-fixed paraffin-embedded specimens or fresh frozen tissue obtained from the primary tumor at diagnosis. RESULTS: Ploidy was evaluable by FCM in all cases, and by ICM in 23/27. When fresh frozen tissue and paraffin-embedded samples from the same tumor were available for analysis, they yielded equal results. The rate of agreement between FCM and ICM was 82%. The majority of cases were diploid, and in the present series aneuploidy seemed to be associated with a poor outcome. CONCLUSIONS: These results suggest that aneuploidy could be an indicator of a bad prognosis in EFT; however, the small number of cases precludes any conclusion of statistical value. Larger retrospective studies on ploidy using archival material could be performed and their reliability is supported by the concordance of results from fresh and formalin-fixed tissue.

Adolescent↗

Langerhans cell histiocytosis arising after Hodgkin's disease.

We present the case of a 19-year-old male who suffered from Langerhans cell histiocytosis (LCH) 12 months after having been treated for recurrent Hodgkin's disease (HD). Immunophenotypic characterization and electron microscopic analysis were useful for the exclusion of a bone relapse of Hodgkin's disease or any other differential diagnosis. The association of LCH with HD or other malignancies is rare but more frequent than previously believed. The significance of such an association and the pathophysiology of LCH are still open questions.

Adult↗

Anaplastic lymphoma kinase (ALK) is not expressed in Hodgkin's disease: results with ALK-11 antibody in 327 untreated patients.

The t(2;5)(p23;q35) or other rare chromosomal abnormalities involving 2p23 upregulate the ALK gene, which is not expressed in normal lymphocytes. Thus, detection of ALK protein is presumptive evidence of these 2p23 abnormalities. The t(2;5) and ALK immunoreactivity are common in anaplastic large cell lymphoma of T/null-cell lineage. However, a small subset of cases of Hodgkin's disease (HD) have been reported to either carry the t(2;5) or express ALK. In this study, we have immunohistochemically evaluated 327 cases of HD with the ALK-11 antibody. ALK-11 is a well characterized polyclonal antibody raised against an intracellular portion of the ALK protein. We detected ALK-11 immunoreactivity in 8 (2.4%) cases of HD. We further studied these positive cases with ALK-1 monoclonal antibody, which reacts with an intracellular portion of ALK, similar to ALK-11. All 8 ALK-11 positive cases were negative for ALK-1. These results indicate that rare cases of HD may react with ALK-11 antibody, similar to previous reports by others using different polyclonal anti-ALK antibodies. However, the absence of ALK-1 expression in these HD cases suggests that ALK protein is not truly present and that polyclonal anti-ALK antibodies may rarely yield non-specific cross reactivity. These results further support the use of anti-ALK antibodies in the differential diagnosis of HD from ALCL.

Adolescent↗

Prognostic value of clinicopathologic characteristics including neuroectodermal differentiation in osseous Ewing's sarcoma family of tumors in children.

AIMS AND BACKGROUND: The aim of the present study was to determine the relationship between clinico-pathologic parameters, including neuroectodermal differentiation, and their impact on survival in a series of pediatric patients with osseous tumors of the Ewing's sarcoma family admitted to the Pediatric Department of the Istituto Nazionale Tumori of Milan. METHODS: Seventy-three patients were enrolled. The variables analyzed were sex, age, site of primary tumor, serum lactate dehydrogenase (LDH) level at diagnosis, involvement of periosseous soft tissues by primary tumor, presence of metastatic disease, status of disease after the treatment plan, as well as the presence of mitoses, morphologic and immunocytochemical neural markers, and neuroendocrine markers in the primary tumor. RESULTS: Neural and neuroendocrine markers were not significantly associated with any of the other parameters. In the univariate analysis, significant risk factors related to unfavorable outcome were elevated LDH, metastatic disease, lack of complete remission after treatment, presence of mitoses and of morphological neural markers; immunocytochemical neural and neuroendocrine markers lacked prognostic value. In the multivariate analysis, only LDH levels and the status of disease following the treatment were retained. CONCLUSIONS: LDH level at diagnosis might be a useful marker to identify different risk levels; neuroectodermal differentiation might have no clear-cut impact on the clinical management of osseous Ewing's sarcoma family of tumors.

Adolescent↗