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Article: X-linked lymphoproliferative disease.

Purtilo, D T

1992 Volume 339, Issue 8788, Page(s) 312

MeSH term(s)	Female ; Genetic Linkage ; Humans ; Lymphoproliferative Disorders/genetics ; Male ; Sex Factors ; X Chromosome
Language	English
Publishing date	1992-02-01
Publishing country	England
Document type	Comment ; Letter
ZDB-ID	3306-6
ISSN	1474-547X ; 0140-6736 ; 0023-7507
ISSN (online)	1474-547X
ISSN	0140-6736 ; 0023-7507
DOI	10.1016/0140-6736(92)91390-t
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Zs.MG 94: Show issues

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Article: X-linked lymphoproliferative disease (XLP) as a model of Epstein-Barr virus-induced immunopathology.

Purtilo, D T

Springer seminars in immunopathology

1991 Volume 13, Issue 2, Page(s) 181–197

MeSH term(s)	Adult ; Herpesvirus 4, Human/pathogenicity ; Humans ; Infectious Mononucleosis/complications ; Infectious Mononucleosis/immunology ; Lymphocyte Activation ; Lymphoma/complications ; Lymphoproliferative Disorders/complications ; Lymphoproliferative Disorders/etiology ; Lymphoproliferative Disorders/therapy ; Male ; Models, Biological
Language	English
Publishing date	1991
Publishing country	Germany
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S. ; Review
ZDB-ID	6177-3
ISSN	1432-2196 ; 0344-4325 ; 0172-6641
ISSN (online)	1432-2196
ISSN	0344-4325 ; 0172-6641
DOI	10.1007/bf00201468
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Article: Dual infections of the immune system in patients with chronic active Epstein-Barr virus infection mimicking chronic fatigue syndrome.

Purtilo, D T

Canada diseases weekly report = Rapport hebdomadaire des maladies au Canada

1991 Volume 17 Suppl 1E, Page(s) 29–32

Abstract: The etiologic bases of CFS are undetermined at the present time. It is very important to distinguish the patients with CFS as defined by the Centers for Disease Control (CDC) case definition of Holmes et al. from patients with physical and laboratory ... ...

Abstract	The etiologic bases of CFS are undetermined at the present time. It is very important to distinguish the patients with CFS as defined by the Centers for Disease Control (CDC) case definition of Holmes et al. from patients with physical and laboratory findings suggesting dual infections and/or underlying immunodeficiency. Particularly fruitful might be a longitudinal immunovirologic study of patients who exhibit CFS following a well-documented viral infection.
MeSH term(s)	Diagnosis, Differential ; Fatigue Syndrome, Chronic/diagnosis ; Fatigue Syndrome, Chronic/etiology ; Humans ; Immune System Diseases/diagnosis ; Immune System Diseases/etiology
Language	English
Publishing date	1991-01
Publishing country	Canada
Document type	Journal Article ; Review
ZDB-ID	1034928-5
ISSN	0382-232X
ISSN	0382-232X
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Article: Opportunistic cancers in patients with immunodeficiency syndromes.

Purtilo, D T

Archives of pathology & laboratory medicine

1987 Volume 111, Issue 12, Page(s) 1123–1129

Abstract: Patients with inherited or acquired immunodeficiency disorders are vulnerable to a broad spectrum of opportunistic infectious diseases, but a narrow spectrum of malignancies. These malignancies--B-cell lymphomas, Kaposi's sarcomas, and squamous cell ... ...

Abstract	Patients with inherited or acquired immunodeficiency disorders are vulnerable to a broad spectrum of opportunistic infectious diseases, but a narrow spectrum of malignancies. These malignancies--B-cell lymphomas, Kaposi's sarcomas, and squamous cell carcinomas--are likely due to failure of immune surveillance to recognize virally transformed target cells. The evidence for this hypothesis is substantial regarding Epstein-Barr virus-induced lymphoproliferative lesions in immunodeficient patients.
MeSH term(s)	Acquired Immunodeficiency Syndrome/complications ; Acquired Immunodeficiency Syndrome/immunology ; Acquired Immunodeficiency Syndrome/pathology ; Herpesvirus 4, Human/immunology ; Humans ; Immunologic Deficiency Syndromes/complications ; Immunologic Deficiency Syndromes/immunology ; Immunologic Deficiency Syndromes/pathology ; Immunologic Surveillance ; Neoplasms/etiology ; Neoplasms/immunology ; Neoplasms/pathology ; Opportunistic Infections/etiology ; Opportunistic Infections/immunology ; Sarcoma, Kaposi/etiology ; Tumor Virus Infections/etiology
Language	English
Publishing date	1987-12
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	194119-7
ISSN	1543-2165 ; 0003-9985 ; 0363-0153 ; 0096-8528
ISSN (online)	1543-2165
ISSN	0003-9985 ; 0363-0153 ; 0096-8528
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Article: Epstein-Barr virus: the spectrum of its manifestations in human beings.

Purtilo, D T

Southern medical journal

1987 Volume 80, Issue 8, Page(s) 943–947

Abstract: ... to monoclonality. This conversion likely results from specific reciprocal chromosomal translocations such as t(8;14 ...

Abstract	Epstein-Barr virus (EBV) infects virtually everyone by adulthood, and a lifelong latency is maintained. It infects children silently, whereas the majority of adolescents have infectious mononucleosis (IM). Children who have IM before 5 years of age are often heterophil negative; EBV-specific antibodies are required for diagnosis. On rare occasions the symptoms of IM may persist in a chronic or recurrent form, and fatal infectious mononucleosis occurs rarely. Depending on the type and degree of immune deficiency and the time the EBV infection occurs in the life cycle, various atypical outcomes can occur. Children with primary immune deficiency can have fatal or chronic IM, malignant B cell lymphoma, virus-associated hemophagocytic syndrome, aplastic anemia, or acquired hypogammaglobulinemia. The various outcomes of the EBV infections are likely governed by the immune response of the individual. The increased frequency of B cell neoplasms in immunodeficient patients is likely due, in part, to EBV. Individuals with acquired immune deficiency disorders such as AIDS or allograft recipients may develop malignant B cell lymphomas which tend to be polyclonal, but which may progress through stages of oligoclonality to monoclonality. This conversion likely results from specific reciprocal chromosomal translocations such as t(8;14), which is seen in Burkitt's lymphoma. Detection of EBV in immunodeficient patients is achieved by EBV-specific antibody studies or isolation of virus by obtaining spontaneous lymphoblastoid cell lines from peripheral blood, isolating virus from throat washings, or identifying EBV genome by molecular hybridization techniques. Prevention of primary immune deficiency by early detection and genetic counseling and monitoring of patients for occurrence of EBV infection may lead to early treatment. Acyclovir and immunoglobulin therapy can be of value in some patients with active EBV infection.
MeSH term(s)	Adolescent ; Antibodies, Viral/analysis ; Burkitt Lymphoma/microbiology ; Child, Preschool ; Herpesviridae Infections/microbiology ; Herpesvirus 4, Human/immunology ; Humans ; Infectious Mononucleosis/microbiology
Chemical Substances	Antibodies, Viral
Language	English
Publishing date	1987-08
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S. ; Review
ZDB-ID	185329-6
ISSN	1541-8243 ; 0038-4348
ISSN (online)	1541-8243
ISSN	0038-4348
DOI	10.1097/00007611-198708000-00003
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Zs.MO 336: Show issues

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Article: Lymphotropic viruses, Epstein-Barr virus (EBV) and human T-cell lymphotropic virus-I (HTLV-I)/adult T-cell leukemia virus (ATLV), and HTLV-III/human immune deficiency virus (HIV) as etiological agents of malignant lymphoma and immune deficiency.

Purtilo, D T

AIDS research

1986 Volume 2 Suppl 1, Page(s) S1–6

Abstract: ... retrovirus, ATLV/HTLV-I has T cell tropism. Clinical descriptions by Burkitt and Takatsuki led to discovery ... by blood transfusion. In normal seropositive persons both viruses infect only 1 in about 10,000 B or T ... ATLV is associated with adult T cell leukemia/lymphoma and smoldering T cell lymphoma. EBV infects ...

Abstract	The ubiquitous, DNA herpesvirus, EBV, has B cell tropism and the geographically restricted RNA retrovirus, ATLV/HTLV-I has T cell tropism. Clinical descriptions by Burkitt and Takatsuki led to discovery of these viruses which infect silently early in life; however, ATLV is also transmitted to a spouse or by blood transfusion. In normal seropositive persons both viruses infect only 1 in about 10,000 B or T cells, respectively. EBV is associated with Burkitt's lymphoma, nasopharyngeal carcinoma, and infectious mononucleosis. ATLV is associated with adult T cell leukemia/lymphoma and smoldering T cell lymphoma. EBV infects polyclonally and is controlled by multiple cellular and humoral control mechanisms. Escape from immune surveillance as in immune deficient African children with malaria, males with x-linked lymphoproliferative syndrome, organ transplant recipients, and AIDS patients permits conversion from polyclonal to oligoclonal and finally, monoclonal malignancy. T cell immune defects permit proliferation of cells which undergo molecular and/or cytogenetic alterations. In contrast to EBV, which is integrated and nonintegrated in B cells, ATLV is monoclonally integrated. Viral transforming proteins and immune suppressive substances are produced. Immune deficiency in silent carriers of ATLV and in those with smoldering ATL suggest that immune surveillance deters emergence of ATL. Prevention of primary infection by vaccination against these lymphotropic viruses, and use of immunotherapy and antiviral drugs may potentially retard conversion of infected B or T cells to monoclonal malignancy.
MeSH term(s)	Acquired Immunodeficiency Syndrome/etiology ; B-Lymphocytes/immunology ; B-Lymphocytes/microbiology ; Deltaretrovirus/pathogenicity ; Deltaretrovirus Infections/complications ; Deltaretrovirus Infections/immunology ; HIV/pathogenicity ; Herpesvirus 4, Human/pathogenicity ; Humans ; Immune Tolerance ; Immunologic Surveillance ; Lymphoma/etiology ; T-Lymphocytes/immunology ; T-Lymphocytes/microbiology ; Tumor Virus Infections/complications ; Tumor Virus Infections/immunology
Language	English
Publishing date	1986-12
Publishing country	United States
Document type	Comparative Study ; Journal Article ; Review
ZDB-ID	605665-9
ISSN	0737-6006
ISSN	0737-6006
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Article: Prevention and treatment of Epstein-Barr virus (EBV)-associated lymphoproliferative diseases in immune deficient patients.

Purtilo, D T

AIDS research

1986 Volume 2 Suppl 1, Page(s) S177–81

Abstract: Prevention of EBV-associated lymphoproliferative diseases in immune deficient individuals is preferred; however, standard therapy for the B cell lymphomas has been successful. Chemotherapy must be given cautiously lest further immune compromise result in ...

Abstract	Prevention of EBV-associated lymphoproliferative diseases in immune deficient individuals is preferred; however, standard therapy for the B cell lymphomas has been successful. Chemotherapy must be given cautiously lest further immune compromise result in opportunistic infections. Recently, Acyclovir has decreased morbidity of patients with acute infectious mononucleosis in immune competent persons. In contrast, immunodeficient patients with X-linked lymphoproliferative (XLP) syndrome do not seem to respond favorably. Hence, a prospective study is underway using prophylactic immunoglobulin containing (EBV)-specific antibodies. The mortality rate is 85% following EBV infection in XLP due to fatal infectious mononucleosis associated with fulminant hepatitis and virus-associated hemophagocytic syndrome, acquired hypogammaglobulinemia or malignant B cell lymphoma. We can detect XLP by noting failure of switching from IgM to IgG antibody production on secondary challenge with bacteriophage phi X174. Also, linkage studies with the XLP locus using restriction fragment length polymorphisms are being done to detect affected males pre-EBV infection. Our rationale for prevention of phenotypes of XLP is based on observations that infants in tropical Africa and males with XLP do not develop EBV-induced diseases while neutralizing maternal antibodies are present. An EBV vaccine will be used, when available, in seronegative males with XLP. Prevention of acquired immune deficiency by screening blood for human immune deficiency virus, encouraging prudent life styles, development of specific immunosuppressive agents, development of new antiviral agents (i.e., DHPG), and identification of high risk seronegative patients offer possibilities for preventing life-threatening EBV-induced diseases.
MeSH term(s)	Acyclovir/therapeutic use ; Genetic Counseling ; Herpesviridae Infections/complications ; Herpesviridae Infections/prevention & control ; Herpesvirus 4, Human/immunology ; Herpesvirus 4, Human/pathogenicity ; Humans ; Immunologic Deficiency Syndromes/etiology ; Immunologic Deficiency Syndromes/prevention & control ; Immunosuppressive Agents/therapeutic use ; Immunotherapy ; Interferons/therapeutic use ; Lymphoma/drug therapy ; Lymphoma/prevention & control ; Lymphoproliferative Disorders/complications ; Lymphoproliferative Disorders/genetics ; Lymphoproliferative Disorders/immunology ; Male ; Prospective Studies ; Viral Vaccines ; X Chromosome
Chemical Substances	Immunosuppressive Agents ; Viral Vaccines ; Interferons (9008-11-1) ; Acyclovir (X4HES1O11F)
Language	English
Publishing date	1986-12
Publishing country	United States
Document type	Journal Article
ZDB-ID	605665-9
ISSN	0737-6006
ISSN	0737-6006
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Article: Epstein-Barr virus-induced diseases in the X-linked lymphoproliferative syndrome and related disorders.

Purtilo, D T

Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie

1985 Volume 39, Issue 2, Page(s) 52–58

Abstract: Information regarding the development of diverse diseases associated with EBV virus in immune deficient patients has been gained by studying males with XLP, and their families. Multiple immune defenses normally protect against the ubiquitous EBV. ... ...

Abstract	Information regarding the development of diverse diseases associated with EBV virus in immune deficient patients has been gained by studying males with XLP, and their families. Multiple immune defenses normally protect against the ubiquitous EBV. Depending on the type and degree of inherited or acquired immunodeficiency, EBV may more or less be capable of inducing a variety of diseases. Multiple methods may be needed to document EBV in the immune deficient individual. Rational approaches to prevention and intervention in EBV-induced diseases in immune compromised individuals are being developed.
MeSH term(s)	Animals ; Female ; Herpesviridae Infections/complications ; Herpesvirus 4, Human ; Humans ; Immunologic Deficiency Syndromes/microbiology ; Lymphoproliferative Disorders/genetics ; Lymphoproliferative Disorders/immunology ; Lymphoproliferative Disorders/microbiology ; Male ; Phenotype ; Risk ; X Chromosome
Language	English
Publishing date	1985
Publishing country	France
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S.
ZDB-ID	392415-4
ISSN	1950-6007 ; 0753-3322 ; 0300-0893
ISSN (online)	1950-6007
ISSN	0753-3322 ; 0300-0893
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Article: Clonality of EBV-induced lymphoproliferative diseases in immune-deficient patients.

Purtilo, D T

The New England journal of medicine

1984 Volume 311, Issue 3, Page(s) 191–192

MeSH term(s)	Child ; Clone Cells ; Herpesviridae Infections/pathology ; Herpesvirus 4, Human ; Humans ; Immune Tolerance ; Immunologic Deficiency Syndromes/complications ; Lymphoproliferative Disorders/pathology
Language	English
Publishing date	1984-07-19
Publishing country	United States
Document type	Letter
ZDB-ID	207154-x
ISSN	1533-4406 ; 0028-4793
ISSN (online)	1533-4406
ISSN	0028-4793
DOI	10.1056/NEJM198407193110314
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Article: Defective immune surveillance in viral carcinogenesis.

Purtilo, D T

Laboratory investigation; a journal of technical methods and pathology

1984 Volume 51, Issue 4, Page(s) 373–385

MeSH term(s)	Acquired Immunodeficiency Syndrome/immunology ; Animals ; Antibody Formation ; Antigens, Neoplasm/immunology ; Burkitt Lymphoma/immunology ; Chromosome Aberrations/immunology ; Chromosome Disorders ; DNA Viruses/immunology ; Deltaretrovirus/immunology ; Herpesviridae/immunology ; Herpesvirus 4, Human/immunology ; Homosexuality ; Humans ; Immunologic Deficiency Syndromes/congenital ; Immunologic Deficiency Syndromes/genetics ; Immunosuppressive Agents/adverse effects ; Lymphoma/chemically induced ; Oncogenic Viruses/immunology ; Polyomavirus/immunology ; Retroviridae/immunology ; Translocation, Genetic ; Tumor Virus Infections/immunology
Chemical Substances	Antigens, Neoplasm ; Immunosuppressive Agents
Language	English
Publishing date	1984-10
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S. ; Review
ZDB-ID	80178-1
ISSN	1530-0307 ; 0023-6837
ISSN (online)	1530-0307
ISSN	0023-6837
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