Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinicopathological analysis was performed in 19 patients diagnosed clinically with malignant histiocytosis. Ultimately, 9 patients died and 10 are still alive. All 19 had fever of unknown origin. Among the 10 surviving patients, 6 recovered with only supportive therapy such as antibiotic treatment. One recovered with steroid therapy and 2 with VP (vincristine and prednisolone) therapy. Complications due to immunodeficiency were detected in one surviving patient and 2 who died. All 9 patients who died had anemia, and 8 had thrombocytopenia. However, among survivors, only one had anemia and only 2 had thrombocytopenia. Chromosomal abnormality was detected in one patient who died. Histiocytic cells were classified morphologically into 3 types: immature, intermediate and mature. In 4 patients who died, histiocytic cells were immature, but in 4 others mature histiocytic cells were detected. In 5 of the 10 surviving patients, histiocytic cells were of the immature type. Immuno-histochemical analysis of the origin of histiocytic cells in 8 deceased patients showed T-zone histiocytes in one, T cells in one, monocyte phagocytic system (MPS) in 5, and histiocytes of unknown origin in one. Thus, malignant histiocytosis is a heterogenous entity including reactive histiocytic disorder, lymphocytic neoplasm and true histiocytic neoplasm. In histiocyte proliferative disorders, red blood cell counts and platelet counts are useful for assessing prognosis, while cytological findings only confuse this evaluation.
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PMID:[Evaluation of clinical features, cytopathological findings and prognosis of histiocyte proliferative disorders]. 140 58

Patients infected with the human immunodeficiency virus (HIV) suffer from serious and life-threatening infections. These patients often present with pyrexia but without localizing signs. Despite its high sensitivity in identifying focal infection 67Ga citrate scintigraphy lacks specificity; lymphoma and solid tumours may also be imaged. This presents particular problems in HIV-positive patients with pyrexia where the differential diagnosis is often between infection and lymphoma. In an attempt to improve the specificity of radionuclide imaging in these patients a combination of 67Ga citrate and the new agent 99Tcm-labelled human immunoglobulin (99Tcm-HIG) was used in 25 patients who were sequentially imaged with the two agents. Fourteen patients had 29 sites of microbiologically confirmed infection; 67Ga citrate identified 27 sites and 99Tcm-HIG identified 16 sites. Seven of the nine sites visualized with 99Tcm-HIG, but positive with 67Ga citrate, were intrathoracic. Abnormal concentration of 67Ga citrate, not due to infection, occurred at eight sites; five lymphoma, one gout, one recent fracture and one patient with prominent bone marrow islands. 99Tcm-HIG showed increased concentration of tracer in only one of the patients with lymphoma. A combination of 67Ga citrate and 99Tcm-HIG imaging in HIV-positive patients with pyrexia of unknown origin enables a differentiation between infection and lymphoma to be made more readily. The poor sensitivity of 99Tcm-HIG in the chest will limit its sole use in this patient group.
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PMID:Combined 67Ga citrate and 99Tcm-human immunoglobulin imaging in human immunodeficiency virus-positive patients with fever of undetermined origin. 192 50

There is a paucity of published information available on extrapulmonary cryptococcosis (EC) in children infected with human immunodeficiency virus, the etiologic agent of the acquired immunodeficiency syndrome. We surveyed investigators in pediatric acquired immunodeficiency syndrome around the country regarding their experience with EC. Investigators from 33 (87%) of 38 institutions responded and information on 13 patients from 11 institutions was analyzed. EC was the acquired immunodeficiency syndrome indicator disease in 9 (69%) of 13 patients. Median age was 8 years with a range of 2 to 17 years. Human immunodeficiency virus risk factors were transfusion (5 patients), hemophilia (4 patients) and perinatal exposure (4 patients). Meningitis, seen in 62% of patients, was the most common clinical manifestation. Although 2 patients with fulminant disease died before therapy was started, 10 (91%) of 11 had a clinical response to amphotericin B with or without flucytosine. Our study indicates a spectrum of EC in pediatric human immunodeficiency virus infection ranging from fulminant, fatal fungemia to chronic meningitis and fever of unknown origin. Cryptococcosis was generally not the cause of death in patients who initially responded to amphotericin B therapy. Optimal antifungal therapy, including the role of fluconazole, warrants further study.
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PMID:Extrapulmonary cryptococcosis in children with acquired immunodeficiency syndrome. 192 78

A retrospective study of tuberculosis was undertaken among 125 patients infected with human immunodeficiency virus (HIV) who attended our regional infectious disease unit between 1986 and 1989. Nine TB-positive patients (five English, three Africans, one Indian) were identified. In three patients who presented with pyrexia of unknown origin and no objective evidence of any organ involvement, the diagnosis of TB was established from examination of sputum induced by nebulized hypertonic saline. Four other patients had extrapulmonary disease while another two had only pulmonary manifestations of TB. Chest radiographs from five patients were normal, while the other four showed cavities with consolidation, pleural effusion, miliary opacities and hilar enlargement, respectively. All but two mycobacterial isolates were fully sensitive to standard first-line chemotherapeutic drugs. Response to treatment was rapid and only complicated in one patient. There were no relapses following treatment without maintenance therapy after a mean follow-up of 22.2 months (range 9-48). Three patients died, of causes unrelated to TB. Tuberculosis may occur at any stage of HIV disease and is an important cause of fever in HIV-infected British patients, even when chest radiographs are normal and previous BCG vaccination has been performed.
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PMID:Tuberculosis in patients infected with the human immunodeficiency virus. 194 41

Hodgkin's disease (HD) was diagnosed in 24 patients who were either seropositive for human immunodeficiency virus (HIV) (21) or members of a high-risk group (three), but had not developed acquired immune deficiency syndrome (AIDS). Clinical presentation of the disease was characterized by constitutional symptoms in all, especially fever (23/24) and disseminated disease (22/24) at diagnosis. Mediastinal adenopathy was rare. Bone marrow involvement was particularly frequent (12/24), and a positive bone marrow biopsy preceded lymph node biopsy in 5 of the 12. Histopathologic features of these tumors included an increased number of nonlymphoid stromal cells, i.e., histiocytic and/or fibroblastoid. In some tumors these fibrohistiocytoid stromal cells were arranged in bundles, but distinct nodule with birefringent collagen band formation was not observed. Twenty-two patients were treated, most with combination chemotherapy; one was untreated; one, unknown. Sixteen, including the one untreated, died with disease at 3 to 25 months; one died of an unrelated cause; four were alive at 3 to 24 months; three were lost to follow-up. Frequent bone marrow involvement at presentation suggests the usefulness of the bone marrow biopsy for diagnosis in subjects at risk, especially when they present with spiking fever of unknown origin. Contrary to most previous series, virtually all of our cases were of mixed cellularity type, characterized by increased fibrohistiocytoid stromal cells in place of depleting lymphocytes. The classic nodular sclerosing feature with birefringent collagen band formation was not observed. In conclusion, HIV-associated HD was characterized by advanced stage with fever at presentation, preponderance of mixed cellularity histologic type with increased fibrohistiocytoid stromal cells, and poor outcome. Hodgkin's disease in AIDS patients presents an intriguing biological model to study the role of stromal histiocytes in immunodeficient patients.
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PMID:Human immunodeficiency virus-associated Hodgkin's disease. Clinicopathologic studies of 24 cases and preponderance of mixed cellularity type characterized by the occurrence of fibrohistiocytoid stromal cells. 172 93

Opportunistic infection with the causative agent of cat scratch disease may be responsible for an unusual vascular proliferative lesion, referred to as bacillary epithelioid angiomatosis, previously described only in human immunodeficiency virus (HIV)-infected patients. We present a case of an HIV-infected patient with bacillary epithelioid angiomatosis involving the liver and bone marrow causing progressive hepatic failure. We also report a case of a cardiac transplant recipient with hepatic and splenic bacillary epithelioid angiomatosis manifesting as a fever of unknown origin, a previously unreported event in a non-HIV-infected patient. These cases represent the first documentation of bacillary epithelioid angiomatosis with visualization of cat scratch-like organisms involving internal organs.
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PMID:Visceral bacillary epithelioid angiomatosis: possible manifestations of disseminated cat scratch disease in the immunocompromised host: a report of two cases. 238 68

Bone marrow examinations were performed on 20 patients with acquired immune deficiency syndrome (AIDS) and 39 with AIDS-related complex (ARC). Fever of unknown origin and thrombocytopenia were common in ARC, but anemia and leukopenia were most frequent in AIDS. Changes in stromal cells and perivascular cuffing of plasma cells were found significantly more often in patients with AIDS than in those with ARC. Malignancies were common in both groups. Human immunodeficiency virus (HIV) nucleic acids were detected with the use of a 3H-labeled cDNA probe with an in situ hybridization method in 11 bone marrow samples (three ARC and eight AIDS). Most commonly positive cells were mononucleated, resembling lymphocytes and histiocytes. Endothelial cells, interdigitating reticulum cells, nucleated red blood cells, and immature myeloid cells also had positive results in some instances. The number of HIV-positive cells was not related to the size of the bone biopsies or the clinical diagnoses. The authors postulate that changes in the peripheral blood and bone marrow of these patients may be related to latent persistent infection with HIV.
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PMID:Bone marrow examination in patients with AIDS and AIDS-related complex (ARC). Morphologic and in situ hybridization studies. 281 12

We have summarized the findings in selected congenital and acquired immunodeficiency disorders that frequently display blood and bone marrow abnormalities. Many other immunodeficiency syndromes were not discussed, as bone marrow abnormalities are infrequently seen in them. Examination of the bone marrow is not usually critical for diagnosing most immunodeficiency disorders. However, it may be diagnostic in the evaluation of fever of unknown origin, to rule out other causes of peripheral cytopenias, and to screen for virus-associated malignancies frequently seen in immune-deficient patients (for example, B cell lymphoma, Kaposi's sarcoma). Examination of the bone marrow is critical in the diagnosis of the virus-associated hemophagocytic syndrome and is often important in evaluating patients with the acquired immunodeficiency syndromes who display hematologic disturbances, such as those being treated with AZT, or those who might have opportunistic infections or malignant neoplasms.
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PMID:Bone marrow findings in immunodeficiency syndromes. 306 23

Visceral leishmaniasis is infrequently reported in immunocompromised hosts; hence, the clinical manifestations and outcome of the disease in these patients are unknown. In a series of 10 patients with visceral leishmaniasis complicating renal transplantation (three), hematologic neoplasms (two), systemic lupus erythematosus (two), or infection with human immunodeficiency virus (three), typical hallmarks of kalaazar such as enlargement of spleen or hyperglobulinemia were absent in three and six patients, respectively. Extensive visceral involvement was noted by biopsies or autopsies in four patients. Diagnosis was made during evaluation for fever of unknown origin. Myriads of amastigotes were seen in bone marrow smears. Measurement of antibodies against Leishmania donovani was positive in each patient tested. Ultimately, three patients died, and chronic infections refractory to treatment developed in two other patients. Visceral leishmaniasis is a potentially fatal infection in immunocompromised hosts. Current antiparasitic therapy frequently fails to eradicate L. donovani from infected tissues.
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PMID:Visceral leishmaniasis in immunocompromised hosts. 333 67

Four previously healthy Danish homosexual men developed Kaposi's sarcoma or opportunistic infections with fever of unknown origin and lymphadenopathy. One patient died of a Pneumocystis carinii pneumonia. Three patients had defective cell-mediated immunity with absent leucocyte interferon production and decreased proliferative response to mitogens and antigens. T lymphocyte helper subsets and natural killer cell activity were reduced. Unstimulated mononuclear cells produced leucocyte migration inhibitor factor. Two patients were sexual partners and three had never been to the USA, where cases of severe acquired immunodeficiency have been reported. Thus, the syndrome must also be suspected in European homosexual men who present with fever of unknown origin, opportunistic infections, or Kaposi's sarcoma.
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PMID:Severe acquired immunodeficiency in European homosexual men. 680 93


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