Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Human peripheral blood monocytes were tested for various functions. It was found that 63--70% of monocytes from 12 normal subjects phagocytized either Staphylococcus epidermidis or latex particles; 28% of Staphylococcus organisms exposed to cells were phagocytized in 1 h and 67% were killed within 2 h; 59% of phagocytizing cells reduced NBT; 77% of endocytosed rabbit gammaglobulin was catabolized in 18 h. In Hodgkin's disease, sarcoidosis and severe pulmonary tuberculosis, phagocytic and bactericidal capacity was decreased in one third to two thirds of cases, while catabolism of gammaglobulin was reduced less often and metabolism was practically unmodified. By contrast, phagocytic and bactericidal capacity were practically normal in the common variable form of agammaglobulinemia, while gammaglobulin was catabolized at a low level in all cases. There was no relationship between the functional disorders of monocytes and alterations of lymphocyte stimulation. These results indicate that mononuclear phagocytes may have intrinsic alterations of functions which can result in deficient defense mechanisms and/or immune response.
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PMID:The functions of human monocytes in normal subjects and in disorders associated with immune deficiency. 88 35

The functions of peripheral blood monocytes (phagocytosis, bacteriolysis, the metabolism, and catabolism of a protein antigen) have been studied in 12 normal subjects and in 23 patients suffering from either primary or secondary immune deficiency. Phagocytosis and bacteriolysis were altered in 1/3-2/3 of patients with either Hodgkin's disease, sarcoidosis or pulmonary tuberculosis, whereas catabolism of the protein antigen were found to be abnormal in practically all cases of agammaglobulinemia. These results show that monocytes may have intrinsic functional abnormalities in some conditions.
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PMID:[Human monocyte function in normal subjects and in certain states of immunologic deficiency]. 100 65

1. -Kveim's test (KT) was carried out on 104 patients for whom the diagnostic of sarcoidosis was suspectid. The positive results of KT correlate with other clinical, radiological and histological criteria. 2. - Two patients with idiopathic agammaglobulinemia (AGG) had positive KT. In two other cases of AGG, KT was negative. Two hypothesis are discussed: a) the association of AGG and infraclinical sarcoidosis favorized by the immunological deficit; b) false positive reactions, which raise the question of the specificity of KT. 3. - Lymphocyte transformation test in the presence of Kveim's antigen (LLT-K) was carried out simultaneously with KT 46 times. Among 30 positive KT only 20 LLT-K were positive. Among 16 negative KT 3LLT-K were positive. LLT-K was the only test carried out on 47 subjects. It was negative among 19 healthy control subjects, 19 allergic subjects and 7 patients suspected of sarcoidosis. LTT-K was positive among 3 patients suspected of sarcoidosis and 1 patient with Hodgkin's disease. 4.- LLT-K like other in vitro tests, presents some advantages over the in vivo tests, in particular the rapidity of response. We found that LTT-K was less often positive when compared with KT. The question of specificity is also raised for LTT-K.
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PMID:[Study on Kveim's test. Positive tests in agammaglobulinemia. Comparison with TTL in presence of Kveim's antigen]. 116 59

Male patients with the X-linked lymphoproliferative syndrome (XLP) have an inherited immune deficiency to Epstein-Barr virus (EBV) infection that results in fatal infectious mononucleosis (IM), acquired hypogammaglobulinemia- or agammaglobulinemia, virus-associated hemophagocytic syndrome, and non-Hodgkin's malignant lymphoma (ML). A clinicopathologic analysis of 17 patients with XLP who developed ML was performed. The median age of the patients at the time of diagnosis was 4.0 years (range, 2-19 years). The median overall survival was 12 months (range, 1-216 months). Eight patients had maternally related male relatives with ML. Other phenotypes of XLP were documented in male relatives of the remaining nine patients. Common presenting symptoms were fever, nausea, vomiting, and abdominal pain. Nine patients had "B" symptoms. All ML occurred at extranodal sites. The intestines, most commonly ileocecal, were involved in 76.5% of the cases. Thirteen patients had localized disease (Stages I and II) and four patients had advanced disease (Stages III and IV). A diffuse histologic pattern of growth was observed in all cases. The distribution of histologic subtypes included small noncleaved (41.2%), large noncleaved (17.6%), immunoblastic (17.6%), small cleaved or mixed cell (11.8%), and unclassifiable (5.9%) ML. Surgical resection, radiation therapy, and chemotherapy resulted in disease-free survivals of up to 192 months in eight patients (median 114 months; range, 12-192 months). Eight of 17 patients (47%) are still alive. A median survival of only 6.0 months (range, 1-12 months) was observed in the nine patients who died. No residual ML was found at autopsy. The small noncleaved subtype had an adverse prognosis (seven of nine deaths versus one of eight survivors; P less than 0.05). Bacterial infection was the major cause of death (seven of nine patients). Characteristics that distinguish ML in XLP from other ML include a maternal family history of XLP, early age of onset, acquired hypogammaglobulinemia, post-EBV infection, and ileocecal involvement.
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PMID:Malignant lymphoma in the X-linked lymphoproliferative syndrome. 381 12

The Epstein-Barr virus (EBV)-induced diseases of males with X-linked lymphoproliferative disease (XLP) include fatal infectious mononucleosis (IM), non-Hodgkin lymphoma (ML), agammaglobulinemia, and aplastic anemia. These phenotypes also occur as sporadic cases in families, and EBV seronegative males in these families must be considered at risk for XLP until they seroconvert normally to EBV. Given that 50% of males inheriting the defective XLP gene die following primary EBV infection, it is vital that they be identified pre-EBV infection. Here we report results using molecular genetic techniques to provide information as to the relative risks of EBV negative males and potential carrier females in ten families wherein a single male had died of IM.
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PMID:Evaluation of families wherein a single male manifests a phenotype of X-linked lymphoproliferative disease (XLP). 825 4

Two related boys who died from fulminant infectious mononucleosis were diagnosed with X-linked lymphoproliferative disease type 1 (XLP-1). Family screening (n=17) identified 6 female mutation carriers and 2 more XLP-1 patients in whom, despite recurrent infections, agammaglobulinemia, and Hodgkin's Disease, the genetic basis had been unknown; demonstrating that awareness and early genetic testing are crucial to reveal underlying primary immunodeficiencies and improve outcome. Furthermore, XLP should be included routinely in the differential diagnosis of severe hypogammaglobulinemia and/or lymphoma in males.
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PMID:Fatal EBV infection and variable clinical manifestations in an XLP-1 pedigree - rapid diagnosis of primary immunodeficiencies may save lives. 2314 65

Recurrent Campylobacter enteritis is a well-recorded complication of primary hypogammaglobulinemia but has only rarely been reported with other types of immunodeficiency, and no cases have been reported after rituximab-associated secondary hypogammaglobulinemia. We therefore reviewed our local microbiology laboratory databases and conducted a literature search, to provide a detailed characterization of the immunodeficiency states associated with recurrent Campylobacter enteritis. Published cases had primary hypogammaglobulinemia, most frequently in the setting of common variable immunodeficiency, x-linked agammaglobulinemia, and Good syndrome. No cases were identified in the literature after rituximab or secondary hypogammaglobulinemia. We report a 73-year-old patient with recurrent Campylobacter enteritis and hypogammaglobulinemia in the setting of non-Hodgkin lymphoma, chemotherapy, and maintenance rituximab. Physicians should be aware of the association of recurrent Campylobacter enteritis and immunodeficiency, most commonly in primary hypogammaglobulinemia. Rituximab alone may not be sufficiently immunosuppressive for recurrent campylobacteriosis to occur; additional factors, including hematological malignancy and its treatment, appear necessary. Patients with recurrent Campylobacter enteritis and those starting rituximab should be investigated for hypogammaglobulinemia and B-lymphopenia.
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PMID:Recurrent Campylobacter Enteritis in Patients with Hypogammaglobulinemia: Review of the Literature. 3208 73