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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A detailed immunologic study of three cases of sinus histiocytosis with massive lymphadenopathy (SHML) was performed to better characterize this rare disorder. One patient had prominent cervical lymphadenopathy that regressed spontaneously, whereas the other two patients had persistent cervical lymphadenopathy and recurrent infections. The first patient was otherwise healthy and had normal immunologic studies. One of the latter patients had a relative increase in blood B cells, a decreased level of serum immunoglobulin A (IgA), decreased blood lymphocyte mitogenic responses to multiple mitogens (37-42% of controls), and cutaneous anergy. The other patient with persistent disease also had a relative increase in blood B cells, polyclonal hypergammaglobulinemia, and circulating immune complexes, as well as decreased blood T cells and markedly decreased blood lymphocyte responses to mitogens (12-37% of controls). Immunohistochemical stains of the lymph nodes of the three patients revealed a characteristic phenotype for the sinus histiocytes: S-100 protein, 3/3; CD14 (Leu M3) 3/3; CD11c (Leu M5), 1/1; CD71 (OKT9), 3/3; CD4 (Leu 3a), 2/3; CD1a (OKT6), 1/3; alpha-1-antitrypsin, 3/3; alpha-1-antichymotrypsin, 3/3; CD35 (C3b), 1/1; CD11b (Mo1), 0/3; CD15 (Leu M1), 0/3; HLA-DR, 0/3; and lysozyme, 0/3. This phenotype suggests that the cells of SHML have features of both the Langerhans/interdigitating cell and mononuclear phagocyte lineages. Emperipolesis by the histiocytes of B cells, T cells, and natural killer cells was demonstrated by a double-staining technique. Our findings indicate that patients with SHML may have a variably expressed immunodeficiency that predisposes them to recurrent infections.
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PMID:Sinus histiocytosis with massive lymphadenopathy: a spectrum of disease associated with immune dysfunction. 171 75

To date, the acquired immunodeficiency syndrome (AIDS) has been identified in over 50 children in the US, including those with associated hemophilia, high-risk environmental factors (Haitian background, parental intravenous drug abuse, or prostitution), and blood transfusions. The evaluation of an infant or young child in whom AIDS is suspected requires exclusion of congenital disorders of immune function. A specific test is not currently available, but inclusion criteria for childhood AIDS have been developed. The diseases accepted as indicative of underlying cellular immunodeficiency children are the same as those used in defining AIDS in adults, with the exclusion of congenital infections such as toxoplasmosis or herpes simplex virus infection in the 1st month of life or cytomegalovirus infection in the 1st 6 months of life. Specific conditions that must be excluded in children are primary immunodeficiency diseases (e.g., DiGeorge syndrome, Wiskott-Aldrich syndrome, ataxia-telangiectasia, neutrophil function abnormality) and secondary immuno-deficiency associated with immunosuppressive therapy, lymphoreticular malignancy, or starvation. Almost all young children with AIDS have hepatosplenomegaly, interstitial pneumonitis, and poor growth. The average age of 36 US child AIDS victims studied in detail was 5 months at presentation with findings suggestive of severe immunodeficiency. Mucocutaneous candidiasis was present in 75% of these 36 children, and Pneumocystis carinii and cytomegalovirus were each isolated from 30% of cases. Normal T4:T8 ratios occur in about 15% of pediatric AIDS cases. Laboratory evidence of polyclonal hypergammaglobulinemia generally supports the AIDS diagnosis. Recurrent infection and malnutrition are major problems in the clinical management of child AIDS patients.
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PMID:Acquired immune deficiency syndrome in childhood. 298 8

Antibody to acquired immunodeficiency syndrome (AIDS)-associated retroviruses (ARVs) was investigated in 68 pediatric patients with abnormalities of T-cell and/or B-cell immunity. All except seven patients conformed to a specific World Health Organization classification for immunodeficiency disease. These seven patients had polyclonal hypergammaglobulinemia and T-cell immunodeficiency. Six of the seven patients had antibody to ARV and had risk factors associated with AIDS. The one patient without antiviral antibody had no AIDS risk factors. No antibody was detected in 61 patients with other primary immunodeficiency disorders. We conclude that ARV first appeared in our population of immunodeficient pediatric patients prior to 1978, is associated with a distinctive immunologic phenotype consisting of polyclonal hypergammaglobulinemia and T-cell immunodeficiency, and does not appear as an opportunistic infection in other immunodeficiency disorders. Detection of the retrovirus associated with AIDS is of value in identifying infants and children who may have unique medical and social problems that occur with AIDS.
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PMID:Antibodies to AIDS-associated retrovirus distinguish between pediatric primary and acquired immunodeficiency diseases. 298 53

The classification of the pediatric acquired immunodeficiency syndrome (AIDS) is based on epidemiologic, immunologic, and virologic data. Persons at risk include mothers who use intravenous drugs, infants who have received blood transfusions from subjects with risk factors, patients receiving factor VIII therapy, and infants born to heterosexual mothers with bisexual husbands. A distinct immunologic phenotype, rarely seen in other immunodeficiency disorders, is associated with pediatric AIDS consisting of polyclonal hypergammaglobulinemia and T-cell immunodeficiency. Detection of antibody to the AIDS retrovirus or isolation of virus are essential in establishing a diagnosis. During early infancy, viral isolation is essential as passive transfer of material IgG may occur. Primary immunodeficiency diseases, in particular adenosine deaminase and purine nucleoside phosphorylase deficiency, should be excluded. A diagnosis of pediatric AIDS may be established in a patient who has a risk factor associated with AIDS, polyclonal hypergammaglobulinemia, T-cell immunodeficiency, and antibody to the AIDS retrovirus or isolation of virus.
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PMID:The acquired immunodeficiency syndrome in infants and children. 299 9

Unique laboratory abnormalities, found in pediatric patients with clinical features of immunodeficiency, led to the original observation that a syndrome of acquired immunodeficiency (AIDS) was also occurring in pediatric populations. Initial observations which demonstrated the nonspecific findings of polyclonal hypergammaglobulinemia and T-cell deficiency were followed by confirmatory findings when testing for the AIDS retrovirus became available. In the pediatric population availability of antibody testing and viral isolation became critical in differentiating primary immunodeficiency disorders which involved both the B- and T-cell systems from AIDS associated with retrovirus infection. At this time based upon clinical, epidemiologic, immunologic, and serologic studies, the syndrome of pediatric AIDS can be distinguished from other primary and secondary pediatric immunodeficiency disorders.
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PMID:Laboratory investigation of pediatric acquired immunodeficiency syndrome. 301 72

Immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma is a distinct peripheral T-cell lymphoma, which closely resembles angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) and/or IBL, but is characterized by focal or sheet-like proliferation of immunoblasts and pale cells of T-cell nature. In this report, 36 patients with IBL-like T-cell lymphoma were analyzed. The disease is clinically characterized by generalized lymph node swelling, hepatosplenomegaly, fever, skin rash, polyclonal hypergammaglobulinemia, marked male predominance, predilection for the elderly, and poor prognosis. There was no association with human T-cell leukemia virus type I or human immunodeficiency virus. IBL-like T-cell lymphoma may be divided into two categories (CD4+ type and CD8+ type) by surface marker analysis. It can also be divided into three categories on the basis of the histologic findings of distribution of morphologically recognizable tumor cells: nine cases of "inconspicuous type," six cases of "patchy type," and 21 cases of "diffuse type." Two cases of "inconspicuous type" converted later to "diffuse type." DNA hybridization analyses in the ten recent cases revealed that three of four "inconspicuous types" and five of six "diffuse types" showed clonal rearrangement of T-cell receptor-beta chain gene without rearrangement of immunoglobulin heavy chain gene, providing strong evidence for clonal proliferation of T cells.
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PMID:Clinicopathologic, immunophenotypic, and immunogenotypic analyses of immunoblastic lymphadenopathy-like T-cell lymphoma. 304 80

Since its first report in 1981, acquired immunodeficiency syndrome (AIDS) has attracted great interest among clinicians. Pediatric cases of AIDS were reported only two years later. Recently a review of the literature revealed about 300 pediatric patients with AIDS who are now tabulated separately by the Centers for Disease Control of Atlanta. The classification of the pediatric AIDS is based on epidemiologic, immunologic and virologic data. Subjects at risk include infants born to intravenous drug-addicted mothers and infants who have received blood transfusions or blood products. The diagnosis of pediatric AIDS may be established in a patient who has a polyclonal hypergammaglobulinemia and T-cell immunodeficiency associated with antibody to human immunodeficiency virus (HIV) or isolation of retrovirus.
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PMID:[AIDS in childhood]. 332 Sep 90

Infection with the human immunodeficiency virus (HIV) leads to selective depletion of the helper/inducer lymphocyte subset and a subsequent state of acquired cellular immunodeficiency. Simultaneously, evidence of B-cell hyper-activity may exist. A subset of patients infected with HIV demonstrates a syndrome of persistent generalized lymphadenopathy (PGL). Lymph node biopsies reveal benign reactive changes with a pattern of florid follicular hyperplasia. A polyclonal hypergammaglobulinemia reflects humoral immune dysfunction. Patients with PGL are similar to those with full-blown AIDS with regards to demographics, immune and virologic studies. Our prospective natural history study of PGL patients initiated in November 1981 reveals a 15% rate of evolution to AIDS in the 200 patient cohort. Factors associated with increased risk of transformation to AIDS include severity of constitutional symptoms, shrinking adenopathy, oral candidiasis or viral hairy leukoplakia, peripheral cytopenias, elevated erythrocyte sedimentation rate or an antecedent episode of herpes zoster. Therapeutic interventions to prevent evolution to AIDS in high risk subsets of lymphadenopathy patients have been investigated. In addition to benign B-cell proliferation associated with HIV infection, malignant lymphomas have also been diagnosed in 29 patients in AIDS risk groups in our clinic population. All patients were male; 26 homosexuals, 2 IV drug abusers and 1 multiply transfused sickle cell anemia patient. Seven patients had antecedent PGL. Non-Hodgkin's lymphoma was diagnosed in 19 patients. Histologies were predominantly diffuse undifferentiated or large cell. Eleven patients were Stage IV at diagnosis. Of 10 patients with mixed cellularity Hodgkin's disease, 7 were Stage IV-B at presentation. Extranodal disease was frequent in patients with lymphomas. Fourteen patients lacked peripheral lymphadenopathy. Response to chemotherapy was good, but complicated by prolonged marrow suppression and development of AIDS-related opportunistic infections. Median survival was 7 months. Laboratory studies investigating the possible role of lymphotropic retroviruses in the development of AIDS-related lymphomas revealed that serum from all patients with high grade non-Hodgkin's lymphoma contained antibodies to HIV and that the majority also expressed antibodies to HTLV-I. This degree of seroreactivity to HTLV-I and HIV was characteristic only of lymphoma patients as sera from only 10 - 15% of AIDS and ARC patients in San Francisco had similar findings.
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PMID:AIDS-related benign lymphadenopathy and malignant lymphoma: clinical aspects and virologic interactions. 382 9

Total serum proteins were evaluated and serum protein electrophoresis performed on 3 500 blood donors after plasmapheresis. The results were standard for total serum proteins, serum albumin and immunoglobulin in 98% of the donors, whereas monoclonal immunoglobulin, immunodeficiencies or polyclonal hypergammaglobulinemia were detected in 2% of them. The frequency of monoclonal components is roughly 0.3%, corresponding to the frequency observed in normal adults of 40 to 60 years old. Donors found with monoclonal immunoglobulins or immunodeficiency should not be considered as eligible for blood donation.
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PMID:[Electrophoretic control of serum proteins in donors designated for plasmapheresis. Initial evaluation of the analysis of 3500 donors]. 402 43

Twenty-seven bone marrow aspirates and biopsy specimens from human immunodeficiency virus-positive patients with plasmacytosis were analyzed to identify the pathologic correlates of polyclonal and monoclonal hypergammaglobulinemia in these patients, to compare the results with another random group of similar human immunodeficiency virus patients with plasmacytosis who did not have serum protein electrophoresis, and to evaluate the significance of the presence of monoclonal proteins in a few patients. Serum protein electrophoresis and immunoelectrophoresis and/or immunofixation electrophoresis revealed monoclonal spikes in five of 18 patients tested. The remaining patients with an abnormal serum protein electrophoresis showed a polyclonal hypergammaglobulinemia. Immunohistochemical stains for kappa and lambda light chains were performed in the bone marrow specimens to determine the presence and/or absence of light-chain preponderance or monoclonality. The percentage of plasma cells varied from 5% to 30% and atypical plasma cells from 1% to 20%. Plasma cell aggregates were present in every case, but variable in number and generally small. In all these cases, including those with monoclonal spikes, plasma cells expressed lambda and kappa light chains with approximately equal intensity. There were no identifiable morphologic differences between the two groups of patients. The paraproteins observed in these patients are likely a reflection of B-cell overactivation. It is important to be aware of this peculiar subset of human immunodeficiency virus-infected patients to avoid an erroneous diagnosis of plasma cell dyscrasia.
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PMID:Plasma cell hyperplasia and monoclonal paraproteinemia in human immunodeficiency virus-infected patients. 848 39


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