PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Immunologic Deficiency Syndromes”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 613 records · Page 34Linked to original sources
PubMed1989

Successful bone marrow transplantation with split lymphoid chimerism in DiGeorge syndrome.

A female infant with DiGeorge syndrome associated with severe T-cell immunodeficiency underwent a successful bone marrow transplantation from her HLA-identical, mixed leukocyte culture-nonreactive brother at 5 months of age. Mature circulating T cells and mitogen-induced proliferative responses were detectable at 10 days posttransplant, and by 8 months post-transplant functional T- and B-cell reconstitution was documented by normal responses to mitogens and normal levels of serum immunoglobulins as well as in vitro and in vivo T-cell reactivity to specific antigens and production of specific antibody to T cell-dependent antigens in vivo. Phytohemagglutinin-induced interleukin-2 production and cell surface interleukin-2 receptor expression improved posttransplant, with normal production values observed by 8 months posttransplant. Histologic examination of appendix and thoracic lymph node obtained 9 and 17 months posttransplant, respectively, revealed near-normal lymphoid architecture, with germinal center formation providing morphologic confirmation of reconstitution. Stable split lymphoid chimerism with T cells of donor origin and B cells remaining recipient in origin was documented by sex chromosome analysis. Two years posttransplant the subject remains free of serious infections. In conclusion, this case indicates that bone marrow transplantation can produce peripheral immunoreconstitution without need for significant thymic influence, most likely by providing a source of postthymic T cells, and that bone marrow transplantation should be considered a therapeutic option in patients with DiGeorge syndrome associated with severe T-cell deficiency.

Bone Marrow Transplantation↗
PubMed1998

[Primary immunologic deficiency by deficiency of HLA class II antigens: nine new Tunisian cases].

BACKGROUND: Bare lymphocyte syndrome is a rare inherited primary immunodeficiency. The majority of the patients reported to date are from North Africa. We report nine new Tunisian cases. POPULATION AND METHODS: Over a period of 5 years, we have established the diagnosis of bare lymphocyte syndrome in nine patients who belong to seven different families. Class II HLA antigen expression was studied on resting peripheral mononuclear cells and PHA blasts. RESULTS: The clinical symptoms started at the mean age of 4.5 months (2-10 months) with chronic diarrhea. The evolution was characterized by appearance of other recurrent infections: pneumopathies (seven cases), thrush (seven cases), otitis (five cases) and septicemia (four cases). Allergic manifestations were observed in four cases. Six patients died at the mean age of 30 months from severe denutrition. Class II HLA antigens were not expressed on resting and activated lymphocytes. The absolute count of TCD4+ lymphocytes was decreased in seven patients. Lymphoproliferative response to specific antigens was absent. Four patients had panhypogammaglobulinemia. CONCLUSION: This study confirms the frequency of this disease among the North African population. The severity of the recurrent infection suggests the diagnosis of bare lymphocyte syndrome. This disease is fatal in the absence of bone marrow transplantation.

Child, Preschool↗
PubMed1986

[Cytostatic agents and defense].

The defence mechanisms of the human body against harmful influences from the environment are manifold. The aggressions on the integrity of the organism are physical, chemical, or biological in nature. In this paper the interactions of the commonly used cytotoxic drugs with various defence mechanisms are discussed. Impairment of these defence mechanisms by cytotoxic drugs results in a whole variety of clinical pictures, such as delayed wound healing, secondary malignant neoplasms or many different infectious diseases. These cytotoxic drug-induced diseases are summarized under the term IDDSS (Induced or Iatrogenic Defence Deficiency SyndromeS).

Antineoplastic Agents↗
PubMed1975

Generalized mastocytosis, relapsing herpes zoster and polyradiculoneuritis.

A 68-year-old woman suffering from a longstanding urticaria pigmentosa with mast cell infiltrations in bones had two attacks of herpes zoster. The second herpes zoster attack was followed by a severe polyradiculoneuritis (Guillain-Barré syndrome). The sequence of events suggests an immunological deficiency.

Aged↗
PubMed1986

Intradermal testing with multiple recall antigens for identification of cell-mediated immune deficiency in homosexual men.

In a health screening project for gay men in Stockholm, delayed cutaneous hypersensitivity was tested in 710 men by a commercial kit (Multitest, Mérieux) containing seven recall antigens and related to past hepatitis B virus (HBV) infection, various life style factors, the lymphadenopathy syndrome (LAS), and antibodies to human T-lymphotropic virus type III (HTLV-III). The multiscore (MS: the sum of all positive reactions) was significantly decreased in men with the following characteristics: HBV markers, greater than 50 yearly sexual partners, regular practice of receptive rectal intercourse, regular sex in gay bathhouses, recent sexual encounters in the US, greater than 50 lifetime exposures to inhaled nitrates, LAS, and positive HTLV-III serology. Anergy to tuberculin (TU) but not to any other antigen was more common in men with greater than 20 yearly partners, regular practice of receptive rectal intercourse, exposure to inhalant nitrates, LAS as well as lesser degree of lymphadenopathy, and HTLV-III seropositivity. HTLV-III antibodies were demonstrated in 61 of 416 (14.7%) men. During the 2-year follow-up four men have developed acquired immune deficiency syndrome. All four had MS less than 10 mm and TU anergy. In a BCG-immunized population Multitest only adds marginal information as compared to intradermal testing solely with TU, but may yield prognostic information in evaluating HTLV-III positive individuals with respect to development of manifest AIDS.

AIDS-Related Complex↗
PubMed1976

Vidarabine therapy for severe herpesvirus infections. An unusual syndrome of chronic varicella and transient immunologic deficiency.

Six patients with severe herpesvirus infections were successfully treated with vidarabine. One patient had a previously undescribed syndrome of chronic cutaneous varicella infection of eight months' duration, associated with transient but complete duppression of lymphocyte response to conconavalin A. Other diagnoses were severe varicella pneumonia, progressive cytomegalovirus pneumonia associated with acute lymphocytic leukemia, herpes simplex encephalitis, severe zoster associated with stage IV lymphoma, and disseminated herpes simplex in a patient receiving high doses of steroids. All patients showed cessation of new lesions or abrupt clinical improvement between days 2 and 4 after initiation of therapy, and all were cured of their clinical infection. Dramatic improvement in all of our patients and the minimal toxicity observed make vidarabine suitable for use in severe herpesvirus infections.

Adolescent↗
PubMed1992

Chronic fatigue syndrome.

A 36-yr-old white female presented with severe fatigue and symptoms consistent with immune deficiency, but was later found to be suffering from chronic fatigue syndrome. This article discusses the diagnostic criteria for this condition. Chiropractic manipulation afforded relief of some symptoms for this patient.

Adult↗