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

Cytokines play a crucial role in the inflammatory and immune responses. The activity of cytokines is counterbalanced by specific inhibitors with some functioning as receptor antagonists. Inhibitors to interleukin 1 and tumor necrosis factor may have therapeutic potential in conditions such as inflammatory arthritis, diabetes mellitus, disseminated intravascular coagulopathy and septic shock. The ability to modulate host defenses with cytokines and cytokine antagonists may also have applications in the fields of transplantation, oncohematology and immunodeficiency.
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PMID:Immunomodulating functions of tumor necrosis factor and interleukin 1 inhibitors. 131 88

The practice of transfusion medicine has undergone substantial change over the last decade. Much of the impetus for the change has come from the isolation of human immunodeficiency virus (HIV) and the linkage of HIV transmission to blood transfusion. The purpose of this paper is to collate and review the literature relating to the indications for blood transfusion and provide recommendations for the appropriate utilization of blood products. Peer-reviewed and published studies and reviews relating to aspects of clinical blood transfusion were identified through computer searches and searching of the bibliographies of identified articles. Emphasis was placed on the literature published within the last decade and particularly in the years 1985-91. Material was chosen which was of proved clinical importance and in which findings were consistent among different investigators or different centres. Less emphasis was placed on material reporting new findings of uncertain clinical relevance or findings that were not consistent with majority reports. It is concluded that the only indication for red cell transfusion is to increase the oxygen carrying capacity of the blood and that an adjustment downwards in the haemoglobin concentration at which blood is transfused (transfusion trigger) from the traditional level of 100 g.L-1 is supported by the physiological and clinical data. Perioperative haemoglobin concentrations of 80 g.L-1 are acceptable in otherwise healthy young patients. The transfusion trigger should be adjusted upwards from this in medically compromised patients and in the elderly (greater than 60 yr). Fresh frozen plasma (FFP) is only indicated when there are documented deficiencies of coagulation factors. Platelet concentrates (PC) are indicated for the treatment of clinical coagulopathy resulting from thrombocytopaenia or platelet dysfunction. Routine or prophylactic administration of either FFP or PC after cardiopulmonary bypass or during resuscitation from haemorrhage is not indicated.
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PMID:Perioperative haemotherapy: I. Indications for blood component transfusion. 139 59

The association of lymphoma and human immunodeficiency virus (HIV) seropositivity in a patient with a rare congenital clotting disorder (Factor X Friuli) is reported. The propositus was a previously asymptomatic HIV-positive 46-year-old man who suddenly presented a rapidly evolving right exophthalmos due to a centroblastic lymphoma, as the presenting symptom of full-blown AIDS. The clinical picture was characterized by unusual site of presentation, advanced stage and fatal course over a short period of time, despite therapy. Bone marrow, but not lymph node or parenchymal involvement, was present. Central nervous system involvement, with an unusual diffuse and massive pattern, and the B-cell origin of the lesion were demonstrated at autopsy. Few reports exist so far about the association of malignancy and HIV in patients with congenital coagulation disorders and this is the first report about a defect other than classic hemophilia.
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PMID:First report of centroblastic lymphoma in a human immunodeficiency virus-positive patient with a rare congenital coagulation disorder (factor X Friuli). 195 Mar 77

Massive transfusion, or the rapid administration of a quantity of blood products that approximates an individual's blood volume, is associated with many potentially lethal complications. If the need for transfusion is immediate, ie, before adequate typing and crossmatching procedures can be completed, O negative RBCs can be given safely in the interim. Hypothermia caused by cold banked blood is aggravated by multiple environmental factors and should be aggressively avoided through the use of heat lamps, warming coils, blankets, and other warming devices. The coagulopathy seen in massive transfusion probably has a mixed etiology involving dilution and consumption of clotting factors and platelets. Although fresh frozen plasma and platelets both play a critical role in blood replacement, deficiencies should be treated with appropriate component therapy dictated by coagulation studies rather than by protocol. Transfusion reactions, the most serious type of which is the hemolytic reaction, may go unrecognized in the bleeding patient in critical condition. Hemolytic reactions can usually be prevented by careful attention to administrative and clerical accuracy. Although the overwhelming majority of the 10 million units of blood transfused annually are uncontaminated, transmission of hepatitis and the human immunodeficiency virus through blood products remains a significant screening problem. Posttransfusion hyperkalemia and acidosis are more likely to be related to inadequate resuscitation from shock than to administration of blood. Citrate toxicity and hypocalcemia are usually self-limiting disturbances. Prophylactic use of calcium chloride is dangerous and unnecessary. The complexity of the conditions necessitating massive transfusion demands frequent reevaluation of multiple laboratory and clinical factors for effective resuscitation and for safe administration of blood.
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PMID:Massive transfusion: complications and their management. 220 22

Previous publications have described thrombotic events with unclear causes in individuals infected with the human immunodeficiency virus (HIV). We stratified the cases of 52 individuals infected with HIV by degree of immunosuppression and the presence of complicating illnesses. Plasma from these individuals was screened for abnormalities that might predispose to thromboses. We found statistically significant differences between patients with CD4 counts < 200/mm3 and those whose CD4 counts were > 400/mm3 in the following: d-dimers, functional protein C, antigenic protein C, total protein S antigen, free protein S antigen, C4b-binding protein (C4b-BP), and von Willebrand antigen (vWD). Free protein S correlated inversely with C4b-BP; vWD directly with total protein S; and protein C inversely with d-dimers. D-dimers were significantly elevated only in immunosuppressed patients with complicating neoplastic/inflammatory disease. We propose that low-grade disseminated intravascular coagulopathy in severely immunosuppressed individuals with HIV and infectious, inflammatory, or neoplastic complications is responsible for depressed protein C, which, together with elevations in total protein S and vWD (markers of endothelial injury), indicates a thrombotic predisposition.
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PMID:Thrombotic tendencies and correlation with clinical status in patients infected with HIV. 748 83

Patients with hemophilia A without human immunodeficiency virus type 1 (HIV-1) infection have lower CD4+ counts and CD4+/CD8+ ratios than controls. This is usually interpreted as a therapy-induced immunodeficiency. Our data re-examine the effect of therapy on peripheral blood mononuclear cell immunophenotypic subpopulations in all congenital clotting disorders. Since late 1985 we have prospectively observed HIV-1 uninfected persons with all types and severity of disorder. Controls were household members without clotting disorders or HIV-1 infection. Analyses of immunophenotype and treatment included a longitudinal random effects model. Compared with controls, age-adjusted CD4+ counts were significantly lower in treated patients (P < .0001) and in patients with all types of clotting disorders who were seldom or never treated (P = .0005). Significantly lower values among both treated and untreated clotting disorder subjects (P < .05) were likewise found for total lymphocytes, several other T-cell subsets, and the CD4+/CD8+ ratio. For most indexes, including the CD4+ count and CD4+/CD8+ ratio, the type of clotting deficiency was not a significant variable. Comparing persons who had no or minimal therapy with those having the most showed increases in CD8+ (P = .0017) and CD20+ CD21- counts (P = .0255), and a lower CD20+ CD21+/CD20+ ratio (P = .0106) in the latter. Controls and persons with clotting disorders differ in CD4+ count. Among those with clotting factor disorders, there is no difference attributable to type of clotting disorder or factor therapy. Large amounts of treatment increased CD8+ and CD20+ CD21- counts, but were not associated with a change in CD4+ count.
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PMID:Effect on lymphocyte subsets of clotting factor therapy in human immunodeficiency virus-1-negative congenital clotting disorders. The Transfusion Safety Study Group. 835 93

Vascular nursing presents constant challenges. The natural history of vascular disease coupled with the complexity of the patient population provides an environment for constant learning. Providing care to these unique individuals demands a thorough knowledge of vascular anatomy and physiology, diagnostic interventions, treatment modalities, multidisciplinary resources, and nursing interventions. This case study explores the multifaceted realm of vascular nursing by examining the hospital course and multidisciplinary plan of care of a 22-year-old man whose hospital course began with a lower extremity deep venous thrombosis and progressed to pulmonary embolus, phlegmasia cerulea dolens, compartment syndrome, sepsis, arterial thrombosis, severe coagulopathy, priapism, laryngeal bleeding/laryngospasm, and subsequent notification of having received a unit of blood from a donor whose human immunodeficiency virus status was later determined to be positive. The intent of this article is not to define a specific means of practice but to share with colleagues the wealth of knowledge that was gained from this experience.
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PMID:Undiagnosed hypercoagulable state: a case study. 870 92

The autopsy findings of 80 human immunodeficiency virus (HIV)-infected adults, who died between 1982-1995, are presented with special emphasis on the risk factor of hemophilia. The study included 23 blood product recipients (hemophiliacs n = 21; non-hemophiliacs n = 2), 34 homosexuals, four intravenous drug abusers, and 19 patients with no known risk factor. Nearly all individuals (93%) showed the late stage of acquired immunodeficiency syndrome (AIDS). Blood product recipients had a significantly lower overall frequency of opportunistic infections (p < 0.05). Homosexuality was associated with the highest overall frequency of opportunistic infections and HIV-associated malignancies, such as Kaposi's sarcoma and malignant non-Hodgkin's lymphoma. Exclusive visceral involvement of Kaposi's sarcoma was frequent, and no decrease of Kaposi's sarcoma was observed during the study period. Pneumocystis infections, atypical mycobacteriosis, and non-Hodgkin's lymphoma showed a significant increase during the last five years (1991-1995) of the observation interval. Opportunistic infections and malignancies were the cause of death in approximately one-half of the patients. In blood product recipients, hepatic failure due to posthepatitic cirrhosis and hemorrhage due to hepatic failure with subsequent coagulopathy and in non-blood product recipients, bacterial bronchopneumonia, and diffuse alveolar damage were additional major causes of death. The data suggest a lower risk for HIV-infected blood product recipients, particularly hemophiliacs, to acquire opportunistic infections and malignant neoplasms.
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PMID:Autopsy findings in patients with human immunodeficiency virus infection with emphasis on the risk factor of hemophilia. 878 Sep 28

The majority of patients receiving plasma-derived clotting factor concentrates between 1970s and the mid-1980s are now hepatitis C positive. The progression of hepatitis C is extremely variable and there is frequently a poor correlation among liver biochemistry, viral load and the stage of liver disease. Liver biopsy remains the only definitive way of staging fibrosis and grading necroinflammatory activity. Concerns have been expressed about the safety of the procedure; however, with modern regimes for the correction of coagulopathy in patients with inherited bleeding disorders, normal haemostasis may be maintained during the peribiopsy period. We performed 21 liver biopsies between 1984 and 1997 on patients with factor VIII (FVIII) or IX (FIX) deficiency and von Willebrand's Disease (VWD). Four had concomitant human immunodeficiency virus (HIV) infection, five were thrombocytopenic and one had a prolonged prothrombin time (PT). Haemostasis was achieved using an intermittent bolus of factor concentrate or continuous infusion regimens. One patient with VWD received Desmopressin (DDAVP). There were no bleeding episodes associated with biopsy. We suggest that liver biopsy is a safe procedure in patients with inherited bleeding disorders when the coagulopathy is fully corrected. It is the only definitive method of staging the extent of fibrosis associated with hepatitis C infection, and it is this that defines prognosis.
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PMID:Liver biopsy in Irish hepatitis C-infected patients with inherited bleeding disorders. 1084 24

Anemia, thrombocytopenia, and neutropenia are common manifestations in patients with human immunodeficiency virus infection that become more frequent and severe with progression from the asymptomatic state to acquired immunodeficiency syndrome (AIDS). Causes of anemia in AIDS include nutritional deficiencies, infection, and marrow suppression by antiretroviral drugs and by the disease itself. Autoimmune hemolysis and blood loss from gastrointestinal lymphoma or Kaposi sarcoma may also contribute. Granulocytopenia may be due to infection, autoimmunity, or bone marrow suppression by drugs or the immunodeficiency virus. Lymphopenia, the classic hallmark of the disease, typically affects T-helper cells first and worsens as the disease advances. Lymphopenia is a result of the direct cytopathic effects of the virus. Thrombocytopenia can occur from antibodies causing an idiopathic thrombocytopenic purpura-like state from bone marrow suppression or from thrombotic thrombocytopenic purpura. A prolonged partial thromboplastin time due to a coagulopathy caused by lupus anticoagulant causing has been described. A variety of malignancies occurs.
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PMID:Hematological Effects of Human Immunodeficiency Virus Infection. 1088 19


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