ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

TheBCR-ABLgene rearrangement, the initial event in the development of chronic myeloid leukemia, primarily produces clonal expansion in CML by blocking apoptosis, a genetically programmed process of autonomous cell death. The mechanism by whichBCR-ABLblocks apoptosis remains unclear, although recent data are beginning to shed light on the signaling pathway. As with other antiapoptotic signals,BCR-ABLinduces cellular resistance to a wide spectrum of cytotoxic antitumor agents. However, apoptosis induced by both cytotoxic T lymphocytes and natural killer or lymphokine-activated killer cells is not blocked byBCR-ABL. A substantial number of patients with chronic myeloid leukemia can now be cured, and the prognosis has improved even for those patients who are not cured. Interferon-oc has emerged as the treatment of choice for patients who do not undergo an allogeneic bone marrow transplantation. The availability of allogeneic bone marrow transplantation has been increased by the ability to find unrelated donors, although graft-versus-host disease remains a major problem. Adoptive immunotherapy with donor lymphocyte transfusions will induce durable remissions and possibly cures in many patients who relapse after allogeneic BMT. Moreover, a number of investigational approaches, especially autologous BMT, appear promising.

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Recent advances in cytogenetics and molecular biology have led to the realization that cancer is a genetic disorder. This finding is especially evident in acute leukemia where the identification of nonrandom cytogenetic abnormalities has paved the way for the discovery and characterization of associated oncogenes and novel mechanisms of leukemogenesis. New insights into the function of these genes and their products are beginning to provide information about their role in normal cell function and leukemogenesis. This article briefly reviews the most recent advances in this exciting field.

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

The development of successful therapy for most children with acute lymphoblastic leukemia can be attributed to sequential clinical studies that show the importance of combination chemotherapy, sanctuary-specific treatment, and supportive care measures. The relative resistance of acute myelogenous leukemia to chemotherapy led to strategies that include dose-intensified chemotherapy and bone marrow transplantation. The improvement in long-term survival for children with acute leukemia has been gratifying but also has been associated with late effects that underscore the need for careful follow-up and for designing risk-adapted therapies.

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Chronic lymphoproliferative disorders are a heterogeneos group of diseases with a highly variable clinical course. In the past few years, important progress has been made in the classification, biology, and therapy of some of these disorders of which B-cell chronic lymphocytic leukemia is not only the most frequent but also the model for their study. Regarding classification, entities that may be confounded with chronic lymphocytic leukemia have been identified (eg, splenic lymphoma with villous lymphocytes, mantle-cell lymphoma in leukemic phase); these diseases should be known and clearly separated from chronic lymphocytic leukemia because their prognosis and treatment is different from that of chronic lymphocytic leukemia. On the other hand, the molecular basis of some of these diseases (eg, the overexpression of thePrad1/CCND1gene in mantle-cell lymphomas, the relationship betweenbcl-2and bax expression in chronic lymphocytic leukemia homeostasis, the role ofp53tumor suppressor gene mutations in chronic lymphocytic leukemia progression) are increasingly well known. Cytokines (eg, tumor necrosis factor-alpha or IL-2, IL-4, and IL-13) also contribute to the pathogenesis of lymphoproliferative disorders by either promoting cell growth or inhibiting apoptosis. In addition, new treatment possibilities (eg, purine analogues, hemopoietic progenitors transplants) are changing the treatment objetives in some of these diseases. Thus, symptoms' palliation is no longer the only realistic aim in the management of patients with chronic lymphoproliferative disorders, but sustained complete remissions and even cures can be achieved.

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Familial neuroendocrine tumors are reviewed. The most dramatic advances have been in the application of molecular genetic techniques to define the affected genes and to develop predictive testing for patients with multiple endocrine neoplasia syndromes. Germline mutations at specific loci of theRETproto-oncogene have been demonstrated in patients with multiple endocrine neoplasia types IIA, IIB, and familial medullary thyroid carcinoma not associated with multiple endocrine neoplasia. This has led to direct DNA testing for these mutations in patients at risk for these syndromes. The approach to predictive testing, diagnosis, and early treatment of these patients is discussed as a model for the approach to hereditary cancers. Linkage testing with DNA markers is still required for patients with multiple endocrine neoplasia type I because the responsible gene has not yet been isolated. Efforts to clarify the etiologies of other familial neuroendocrine tumors not associated with multiple endocrine neoplasia continue. Familial pheochromocytoma, neuroblastoma, and carcinoid also are reviewed. The use of molecular genetic techniques as a powerful tool for the early identification and treatment of susceptible individuals is emphasized.

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

This review focuses on recent developments in the treatment of pituitary adenomas. Current treatments include both transcranial and transsphenoidal surgery, the medical treatment specifically for endocrinopathies, and finally, radiation treatments including conventional radiation as well as stereotactic radiosurgery. All these modalities are reviewed.

ISSN:1040-8746    

出版商:OVID    

年代:1997

数据来源: OVID