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1994-08-20
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Document 0585
DOCN M9480585
TI Lymphoproliferative disorders and other tumors complicating
immunodeficiencies.
DT 9410
AU Filipovich AH; Mathur A; Kamat D; Kersey JH; Shapiro RS;
Immunodeficiency Cancer Registry, University of Minnesota,; Minneapolis.
SO Immunodeficiency. 1994;5(2):91-112. Unique Identifier : AIDSLINE
MED/94305533
AB Lymphoproliferative disorders and selected carcinomas which occur as
complications of primary or secondary immunodeficiencies are frequently
fatal. The incidence rates of these cancers vary from 1% to as high as
25% among specific groups of persons with primary
(genetically-determined) immunodeficiencies as well as acquired
immunodeficiencies, including immunosuppressed organ transplant
recipients and individuals infected with HIV. Lymphoproliferative
disorders including Epstein Barr virus (EBV) associated B cell
lymphoproliferative disease (BLPD) and Hodgkin's disease represent the
predominant category of tumors in both primary and acquired
immunodeficiencies. EBV is an important cofactor common to many, but not
all, B cell lymphomas. Immunodeficient individuals who are at risk for
developing EBV BLPD may demonstrate both inadequate immune responses to
the virus as well as generalized immunoregulatory dysfunction reflected
as imbalances in cytokine production favoring the proliferation of
transformed B lymphocytes. Historically, the success of treatment of
lymphoproliferative disorders in immunodeficiencies with conventional
multi agent chemotherapies and/or radiation has been limited by
unfavorable tumor response rates and high morbidity and mortality
related to intercurrent opportunistic infections. With improvements in
supportive care and the use of recombinant biologic response modifiers
such as alpha interferon and/or other immunotherapies to treat EBV BLPD,
survival of immunodeficient hosts following tumor diagnosis may improve.
In addition to lymphoproliferative disorders, patients with congenital
immunodeficiencies associated with IgA deficiency (including ataxia
telangiectasia and Common Variable Immunodeficiency) are at increased
risk for gastrointestinal carcinomas. Early detection and surgical
excision of such tumors can result in prolonged survival in such
patients.
DE Comparative Study Human Immunologic Deficiency
Syndromes/*COMPLICATIONS/PREVENTION & CONTROL/THERAPY
Immunosuppressive Agents/THERAPEUTIC USE Incidence Lymphoproliferative
Disorders/*ETIOLOGY/PREVENTION & CONTROL/ THERAPY
Neoplasms/*ETIOLOGY/PREVENTION & CONTROL/THERAPY Risk Factors JOURNAL
ARTICLE REVIEW REVIEW, TUTORIAL
SOURCE: National Library of Medicine. NOTICE: This material may be
protected by Copyright Law (Title 17, U.S.Code).