Important note: Information in this article was accurate in 1989. The state of the art may have changed since the publication date.
PEDIATRIC ONCOLOGY: LYMPHOPROLIFERATIVE DISORDERS
Principles and Practice of Pediatric Oncology. Pizzo PA, Poplack DG, eds. Philadelphia, Lippincott, p. 477-90, 1989.. Unique Identifier : AIDSLINE ICDB/89655602 Seibel NL; Cossman J; Magrath IT; George Washington Univ. Sch. of Medicine and Health Sciences,; Washington, DC
Abstract:
Lymphoproliferative disorders represent a heterogeneous array of diseases involving B-cell proliferations that range from reactive polyclonal hyperplasias to true monoclonal malignant lymphomas. The present chapter focuses on those lymphoproliferative disorders in which an underlying condition leads to a defect in the host, thereby predisposing, or permitting, the expansion of lymphoid cell populations that would be more strictly regulated in the normal individual. These conditions are not usually self-limited and may consist of inherited, iatrogenically induced, or acquired immunodeficiencies. Defects in immunoregulation and immune surveillance may be similar in these conditions. As a result of these defects, particularly in regulation, an increased pool of a subpopulation of lymphocytes may be present, an unchecked proliferation of the subpopulation may occur, and, within this setting, new irreversible cytogenetic aberrations may take place. Further details are presented under the following headings: epidemiology and pathogenesis (inherited, iatrogenically induced, and virally induced immunodeficiencies), pathology, molecular biological aspects of lymphoproliferative disorders, clinical presentation (inherited and iatrogenically induced immunodeficiencies, virally induced clinical syndromes), lymphoproliferative disorders infrequently encountered in pediatrics (angioimmunoblastic lymphadenopathy with dysproteinemia, lymphomatoid granulomatosis, sinus histiocytosis with massive lymphadenopathy, giant lymph node hyperplasia, heavy chain diseases, ocular adnexal lymphoid proliferation, Sjogren's syndrome), differential diagnosis, diagnosis, prevention and treatment, and future directions. The subsection on inherited immunodeficiencies in relation to epidemiology and pathogenesis considers ataxia-telangiectasia, Wiskott-Aldrich syndrome, combined immune deficiencies, and X-linked lymphoproliferative syndrome. The subsection dealing with virally induced immunodeficiencies in relation to epidemiology and pathogenesis covers Epstein-Barr virus and retroviruses. The subsection on virally induced clinical syndromes in relation to clinical presentation considers fatal infectious mononucleosis and HIV (human immunodeficiency virus type I) and human T-cell leukemia virus type I (HTLV-I) infections. (170 Refs)
Keywords: Acquired Immunodeficiency Syndrome/COMPLICATIONS B-Lymphocytes/PATHOLOGY Child Diagnosis, Differential Human Lymph Nodes/PATHOLOGY Lymphoproliferative Disorders/GENETICS/PATHOLOGY/*THERAPY Precancerous Conditions/THERAPY Prognosis Risk Factors Tumor Virus Infections/THERAPY MONOGRAPH REVIEW, TUTORIAL REVIEW
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Important note: Information in this article was accurate in 1989. The state of the art may have changed since the publication date.
