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F Cabanillas

Publications and source records attributed to F Cabanillas.

280 records · Page 16Linked to original sources

Long-term results of treatment of patients with follicular lymphomas.

The management of patients with follicular lymphoma is controversial, particularly in those with follicular small cleaved cell (FSCC) or nodular poorly differentiated lymphoma (NPDL). Some advocate no treatment until symptoms arise, while others prefer to treat with intensive combination regimens and others with single agents. We reviewed the long-term effects of two types of combination chemotherapy on relapse-free survival and survival on 91 patients (88 were stages III-IV) who had received no previous chemotherapy. Those patients with FSCC lymphoma obtaining a complete remission had a significantly longer median survival than did those obtaining a partial remission (13 years versus 2 years). An adriamycin-containing regimen appeared to be the key factor in obtaining a complete remission in FSCC after taking into consideration prognostic factors. Not only was the complete response rate significantly higher for patients receiving adriamycin, but so too was the median survival and relapse-free survival. BCG immunotherapy appeared to be important in preventing relapse in some of the complete responders with NPDL who received adriamycin.

Antineoplastic Combined Chemotherapy Protocols↗

A review and interpretation of cytogenetic abnormalities identified in Hodgkin's disease.

The nature of the cell which gives origin to Hodgkin's disease remains unclear after years of intensive investigation. Cytogenetic data, which are very scarce in Hodgkin's disease, could contribute information which might help elucidate this problem. Review of our own data and others shows that the most frequent abnormalities in this disorder involve chromosomes 14q in 35 per cent of cases, 11q in 32 per cent, 6q in 32 per cent, and 8q in 18 per cent. The most common breakpoint in chromosome 14 occurred at 14q32 where the IgH chain genes reside thus suggesting that in these cases the cell of origin might be a B lymphocyte. The 11q and 6q structural abnormality have also been frequently seen in lymphoid disorders such as ALL and large cell lymphoma. Of interest also is the fact that in a certain type of childhood pre-B cell acute lymphoblastic leukemia which shows cytogenetic abnormalities on 11q23, aberrant myeloid and monocytic markers are seen. This suggests that in Hodgkin's disease a similar phenomenon might occur which could help to explain why the Reed-Sternberg cell expresses myeloid-monocytic antigens such as Leu M-1.

Chromosome Aberrations↗

Philadelphia chromosome without breakpoint cluster region rearrangement in a case of Lennert's lymphoma of suppressor phenotype.

A 60-year-old woman presented with diffuse lymphadenopathy. Diagnostic and staging work-up showed that the patient had diffuse small cleaved cell lymphoma (diffuse poorly differentiated lymphocytic lymphoma) with associated histiocytes (lymphoepithelioid cell lymphoma) by the Kiel classification system. Immunohistologic staining showed a T suppressor cell tumour phenotype. Cytogenetic studies revealed the Philadelphia chromosome (Ph1). On DNA studies, the breakpoint cluster region (BCR) gene was not rearranged suggesting that the Ph1 involvement was not identical to that seen in chronic myelogenous leukemia (CML). This case is presented because of the rarity of Ph1 in lymphoid malignancies, particularly in those of T-cell origin, and because of its potentially adverse implications.

Antigens, Differentiation, T-Lymphocyte↗

Chromosomal abnormalities in adult non-endemic Burkitt's lymphoma and leukemia: 22 new reports and a review of 148 cases from the literature.

Tumour cell karyotypes from patients with Burkitt lymphoma (BL) or Burkitt's type leukemia (ALL3) were studied for correlation with survival, bone marrow and cerebral spinal fluid involvement (CSF), human immunodeficiency virus (HIV) serology, and for recurrent cytogenetic abnormalities. The records of 22 patients with BL from our institution and of 148 cases of BL and ALL3 reported in the literature with karyotypes were evaluated for clinical and cytological features. Overall survival was only 28 per cent and 88 per cent of deaths occurred within the first nine months after diagnosis. Those who survived at least 18 months were unlikely to relapse. Age and gender did not significantly affect survival. Patients presenting with advanced Ann Arbor stage, bone marrow or CSF involvement had lower survival rates. The association of translocations involving chromosome band 8q24 with this disease is confirmed. Sixty-two per cent of karyotypes had t(8;14)(q24;q32) translocations; the recognized variant translocations t(8;22)(q24;q11) and t(2;8)(p12;q24) affected 12 per cent and 9 per cent respectively. Seventeen per cent had abnormal karyotypes but no classic translocation. Patients with variant translocations had the poorest survival rates, and those with the classic t(8;14)(q24;q32) did the best. Despite a small sample size, the variant translocation t(8;22)(q24;q11) appeared to occur at an increased frequency in the patients with AIDS. In the entire group, recurrent involvement of chromosome regions 1q2, 6q11-14 and 17p1 suggests that alteration of genes at these loci, B Cell Growth Factor (BCGF) at 1q2 and p53 on 17p, may contribute to the development and progression of this tumour. Similarly, the frequent trisomies of chromosomes 7, 8, 12 and 18 may indicate an effect on tumour cell growth due to increased gene dosage. Trisomy 12 was found in eight tumours, five from patients with AIDS, suggesting that chromosome 12 has a site or gene whose allelic dosage is selected for in AIDS related lymphoma cells. Cytogenetic studies of adult Burkitt lymphoma and leukemia suggest several likely loci for gene alterations that in conjunction with myc translocations can lead to tumorigenesis.

Acquired Immunodeficiency Syndrome↗

Primary mediastinal large cell lymphoma.

PURPOSE: Primary mediastinal large cell lymphoma PMLCL is a recently described entity which has provoked considerable interest lately. The unique immunophenotype of the malignant cell, as well as conflicting reports regarding its response to therapy, prompted us to review the current literature focusing on the pathological and clinical aspects of this disease. DESIGN: We reviewed the current literature that contained details on the clinical presentation, pathological profile and clinical outcome. RESULTS: Most reports agree that PMLCL typically affects a young female population and presents with symptoms related to compression of mediastinal structures. Pathologically, the malignant cells may arise from a distinctive thymic medullary B cell subpopulation which is characterized by the following phenotype: CD19+, CD21-, CD20+, CD22+. Alterations of c-myc and lack of expression of HLA class 1 antigens on the tumour cell surface have been described. Response to treatment and clinical outcome have varied from one series to another and is possibly explained by the small number of patients in most series and by the heterogeneity of therapy. In general, the outcome does not appear to be worse than that of other large cell lymphomas although, given the young age of these patients, it might be expected to be more favourable. CONCLUSIONS: Primary mediastinal large cell lymphoma is a distinct clinico-pathological entity characterized by: (1) a predominance of young adults; (2) female predominance (female/male ratio of 2:1) in contrast to the other large cell lymphomas; (3) CD19+/CD21- immunophenotype; (4) absence of HLA-class 1 antigen expression; (5) possible involvement of c-myc; (6) frequent dissemination at relapse to unusual anatomic sites in a pattern reminiscent of Burkitt's lymphoma. The role of radiotherapy and the optimal treatment for this disorder are issues that remain unresolved.

Antineoplastic Combined Chemotherapy Protocols↗

Outcome of Hodgkin's disease in elderly patients.

The outcome of Hodgkin's disease, in elderly patients is a subject of controversy. We analysed retrospectively 31 cases of patients older than 60 years that received treatment according to current protocols. We observed a CR rate of 87 per cent and survival of 58 per cent with a median follow-up of 58 months which is clearly better than most reported results in this age group. Although our population consists of only those entered on protocols it suggests that patients whose condition is adequate enough to allow them to receive standard therapy with a curative intent, have a similar outcome to that seen in younger patients.

Aged↗

Simultaneous presentation of hairy cell leukemia and follicular small cleaved cell lymphoma in a patient with previous diagnosis of renal cell carcinoma.

Hairy cell leukemia is a lymphoproliferative disorder of the B lymphoid system that is associated with an increased incidence of second malignancies. Most of these second neoplasma have been solid tumours and few reports of lymphoid malignancies exist. We report here a case of simultaneous presentation of hairy cell leukemia diagnosed in the spleen of a patient under therapy for follicular lymphoma who had an antecedent history of renal cell carcinoma. The hairy cell leukemia and the follicular lymphoma were studied by means of molecular techniques and they were found to contain two different clonal B cell proliferations. This suggests an independent origin of these two B cell neoplasms. As far as we know, this is the first report of the coexistence of hairy cell leukemia and follicular lymphoma.

Carcinoma, Renal Cell↗

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↗

Transformation of follicular lymphoma. Expression of p53 and bcl-2 oncoprotein, apoptosis and cell proliferation.

Transformation in follicular lymphoma represents an abrupt transition in tumor biology. The protein product of the bcl-2 oncogene is overexpressed in most follicular lymphomas and inhibits apoptosis. The protein product of the p53 oncogene prevents cell proliferation and induces apoptosis and is overexpressed in the dysfunctional (mutated) form. We studied 15 transformed lymphomas (large cell type), 7 follicular lymphomas before transformation and 2 control groups of follicular center cell lymphomas with no evidence of transformation (9 small cleaved cell and 10 de novo large cell lymphomas). High p53 expression (> or = 45% cells) was detected by immunostaining in 9/15 transformed lymphomas as compared with 0/10 de novo large cell lymphomas and 1/7 follicular lymphomas. Overexpression of bcl-2 (> or = 50% cells) was similar in transformed (11/15) and de novo large cell lymphomas (6/10). The apoptotic index was high (> or = 2%) in all transformed and large cell lymphomas and low in all follicular lymphomas and in the control group of small cleaved cell lymphomas. The apoptotic index in the transformed lymphomas appeared to be independent of bcl-2 expression and was sometimes paradoxically high in the presence of both p53 and bcl-2 overexpression. The apoptotic index was weakly associated with bcl-2 expression in de novo large cell lymphomas. Expression of p53 did not correlate with proliferation index. Our results indicate that p53 overexpression is a specific step associated with transformation and may occur shortly before it. This is not seen in de novo large cell lymphoma. Furthermore, the high apoptotic index in transformed lymphomas often occurs despite overexpression of bcl-2 and p53, implying that other factors may induce apoptosis in these tumors.

Apoptosis↗