Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors investigated the dependence of the results of the crossover test with thrombocytes and lymphocytes of the donor and serum of the recipient on the rise of thrombocytes 1 and 24 hours following administration of a thrombocyte concentrate. The clinical condition of the patient at the time of thrombocyte administration was taken into account. The donor of thrombocytes isolated in a blood element separator was selected from a panel of cca 800 subjects with previously assessed HLA antigens. A total of 36 administered concentrates was evaluated. From the assembled findings it may be concluded that the result of thromboconcentrate transfusion is significantly influenced by adverse clinical factors in the patient such as splenomegaly, haemorrhage, febrile conditions. A positive crossover lymphocytotoxic test can predict reliably the ineffectiveness of thrombocyte administration. A positive crossover test with the donor's thrombocytes does not affect significantly the success of the transfusion. It seems thus that for the fate of administered thrombocytes the presence of HLA antibodies is decisive and not the presence of specific thrombocytic antibodies.
 ...
PMID:[The significance of the thrombocyte crossmatching test]. 207 27

Refractoriness is a complication of multiple platelet transfusions in 30-70% of patients with bone marrow failure. The major causes are HLA alloimmunisation and non-immune platelet consumption; the latter is usually found in patients with DIC, septicaemia or splenomegaly. Initial management of alloimmunised patients who are refractory to platelet transfusions from random donors is the use of HLA-matched platelet donors; this results in improved responses to platelet transfusions in about 65% of these patients. Platelet crossmatching may reveal the presence of platelet-specific antibodies in some patients who are refractory to platelet transfusions from HLA-matched donors and may assist in the selection of compatible platelet donors. The identification of compatible donors is not possible in all refractory patients; alternative approaches such as plasma exchange and high dose intravenous gammaglobulin have been used in such patients with variable results. Insights into the mechanism of HLA alloimmunisation have suggested methods for its prevention. Primary HLA alloimmunisation is dependent on the presence in transfusions of contaminating cells bearing HLA class II antigens; pure platelet concentrates are non-immunogenic as platelets only express HLA class I antigens. Studies using leucocyte-poor blood components for multitransfused patients have demonstrated a reduction in HLA alloimmunisation from about 50-20% and a decrease in the incidence of refractoriness. Improvements in the techniques for leucocyte depletion of red cell and platelet concentrates and the possibility of inactivation of the HLA class II antigen-bearing cells by UV irradiation might make prevention of alloimmunisation an attainable goal in the near future.
 ...
PMID:Platelet transfusions: the problem of refractoriness. 218 45

Still's disease is a seronegative arthritis of children which, in a limited number of cases, can affect adults. The diagnosis of adult-onset Still's disease is characterized by high fever, arthritis and negative serologic tests for rheumatoid factor and antinuclear antibodies and by at least two minor symptoms (leukocytosis, evanescent rash, serositis, hepato- or splenomegaly, and lympho-adenopathy). Since many diseases present analogous manifestations and the adult-onset Still's disease is generally diagnosed by exclusion, we report two patients, aged 26 and 39, with Still's disease, the former with a classic clinical feature, the latter with a clinical feature characterized by severe hepatic abnormalities. The determination of histocompatibility antigens can be useful because some of them (HLA-DR4 in case 1 and HLA-DRw6 in case 2) are frequently associated with the adult-onset Still's disease. The role of anti-inflammatory therapy (acetylsalicylic acid, indomethacin, steroids) must be emphasized, whose efficacy can constitute the pathognomonic element on which the diagnosis of adult-onset Still's disease can be based in a proper clinical pattern.
 ...
PMID:[Still's disease in adults: diagnostic problems]. 224 94

Some enlarged spleens do not seem to be related with known pathogenetic mechanisms (passive congestion, functional workload, malignant infiltration and inflammatory or storage disorders). Non-tropical idiopathic splenomegaly (Dacie's syndrome) is a form of hypersplenism of unknown origin that evolves into a non-Hodgkin lymphoma, after a variable interval, in 20% of the patients. Tropical idiopathic splenomegaly (or hyperreactive malarial splenomegaly) develops when a chronic malarial challenge triggers an abnormal immunological response consisting in decreased suppressor T lymphocytes and increased amounts of circulating immunoglobulin M and immunocomplexes, which are cleared by the splenic macrophages. This peculiar response to malaria seems to be linked to particular HLA antigens. Other confusing splenomegalies are seen in Felty's syndrome, in populations subjected to recurrent infections, and in some families. Overlapping findings and diseases suggest chronic antigenic stimulation as a common feature, with diverse responses depending on the host. A small percentage (probably less than 3%) of normal individuals has minimal splenomegaly without any clinical significance.
 ...
PMID:[Dacie syndrome and other splenomegalies without apparent cause]. 226 48

We examined the iron status of 23 adult patients with hemoglobin H (Hb H) disease. None of them had received multiple blood transfusions or prolonged iron therapy. Studies included serum iron and ferritin concentrations, transferrin saturation, a desferrioxamine test, computed tomography (CT) scan of the liver, and liver biopsy. Iron overload was found in 17 patients (73.9%), especially in males and in patients with splenomegaly (92.9% and 100%, respectively). Four patients with excessive alcohol consumption had clinical manifestations of severe iron overload. Idiopathic hemochromatosis associated HLA antigens, i.e., HLA-A3, -B7, or -B14, were not found in any of the 15 patients tested. These findings indicate that iron overload is common in adult patients with Hb H disease; such patients should abstain from alcohol and be considered for treatment with an iron chelating agent before irreversible organ damage occurs.
 ...
PMID:Iron overload in Chinese patients with hemoglobin H disease. 236 95

Immune and nonimmune causes of platelet refractoriness were evaluated in a group of patients receiving HLA-selected single-donor platelet transfusions. During a 1-year observation period, 1 h and 24 h platelet recoveries wre determined after 522 single-donor platelet transfusions given to 43 patients persistently refractory to pooled random-donor platelet transfusions. 72% of patients tested ultimately developed lymphocytotoxic antibodies suggesting they were alloimmunized. When significant lymphocytotoxic antibodies were demonstrable in these patients, HLA well-matched platelet transfusions consistently produced good transfusion responses. In contrast, patients without lymphocytotoxic antibodies had clinical factors that adversely affected transfusion outcome (P less than 0.0001). Fever and splenomegaly markedly reduced 1 h post-transfusion platelet recoveries, while sepsis compromised the 24 h platelet recovery. Overall, the presence of any clinical factor was most likely to reduce 1 h platelet recovery, while donor-recipient HLA incompatibilities correlated best with poor 24 h post-transfusion platelet recovery. A platelet crossmatch test predicted the transfusion response when non-immune clinical factors were absent.
 ...
PMID:Factors influencing the transfusion response to HLA-selected apheresis donor platelets in patients refractory to random platelet concentrates. 260 25

Multiple lymphomatous polyposis of the gastrointestinal tract was initially described as mucosal lymphomatous involvement by any of a variety of Hodgkin's or non-Hodgkin's lymphomas that produced a polypoid appearance over long segments of the gastrointestinal tract. We studied four patients in whom histology revealed diffuse small cleaved cell lymphoma (one case), or intermediate lymphocytic lymphoma of diffuse type (one case), or mantle zone pattern (two cases). All four cases are classifiable as centrocytic lymphoma. Cell suspension and immunocytochemical studies demonstrated B-cells of IgMD or M type with light chain restriction (two kappa, two lambda) showing a B1+ HLA Dr+ LN2+ CD5+ CD10+. Although all four patients had a partial response to combination chemotherapy, three of them died within 3 years. Analysis of 24 cases reported since 1971 (including the present cases) suggests that MLP is a distinct clinicopathological entity that results from gastrointestinal involvement by a B-cell centrocytic lymphoma. It is distinct from the recently described clinicopathological forms of centrocytic lymphoma and intermediate lymphocytic lymphoma, which both show extensive peripheral lymphadenopathy and splenomegaly, but it is probably closely related to them. The differences are probably attributable to distinct cell tropism or homing properties rather than to cellular histogenesis or degree of maturation.
 ...
PMID:Multiple lymphomatous polyposis of the gastrointestinal tract. A clinicopathologically distinctive form of non-Hodgkin's lymphoma of B-cell centrocytic type. 266 36

The mouse monoclonal antibody 25-3 specific for the alpha subunit of LFA-1 (CD11a) has been infused to children undergoing HLA non-identical bone marrow transplantation because of lethal inherited diseases or of leukemia in order to prevent graft failure. We have assessed the serum concentrations of the antibody infused according to two regimens: 0.1 mg/kg five times, every alternate day (eight patients) or 0.2 mg/kg daily for 10 days (30 patients). Serum trough levels of 25-3 antibody in the first group were constantly found to be low (less than 0.6 micrograms/ml) while 25-3 serum concentration in the second group rose progressively to a mean value of 2.2 micrograms/ml. Serum antibody concentrations were significantly lower in patients with greater antigenic mass, i.e. with splenomegaly, or non conditioned. The engraftment rate was slightly higher in patients treated by the daily infusion of 25-3 for 10 days. No immunization against 25-3 antibody occurred. One patient who subsequently received other mouse anti-B cell antibodies eventually produced anti-isotype antibodies. The consequences of 25-3 infusion on leukocyte counts has been evaluated in the group of patients (n = 6) with severe combined immunodeficiency who did not receive any chemotherapy and in one who was treated with 25-3 because of acute graft-versus-host disease. In none of the cases did 25-3 antibody infusion modify blood leukocyte counts. In addition, it was shown that saturation of LFA-1 on leukocytes was a transient phenomenon, since it could not be found 24 h following infusion of 25-3 antibody.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:In vivo infusion of anti LFA-1 antibody in HLA non-identical bone marrow transplantation in children: serum concentrations and biological effects. 267 57

Hyperreactive malarious splenomegaly (HMS) represents an abnormal immune response to recurrent malaria, characterized by excessive production of both IgM and IgG antibodies. It has both a racial and a familial distribution in various parts of the world. Immune responses to many foreign antigens, including those of malaria, are under genetic control of the major histocompatibility locus (MHC), through the influence of HLA antigens on regulatory T-lymphocyte activity. It is therefore likely that this region also contains the genetic determinants for HMS, which would be reflected in associations between HMS and particular HLA antigens or haplotypes. Genetic studies of the Watut people of Papua New Guinea have not shown any association between HMS and a wide range of red cell and serum polymorphisms. However, HMS in this group is associated with the class II HLA antigen DR2, and with high levels of HLA heterozygosity. Formal genetic analysis of family data also points to a sex-linked gene as a further determinant of overresponsiveness to malaria in the Anga. These findings suggest that more than one genetic system may be involved in the development of HMS, and that the combined effects of several genetic determinants may be responsible for the extraordinarily high frequency of HMS found in the Upper Watut Valley.
 ...
PMID:The genetic basis of hyperreactive malarious splenomegaly. 269 22

Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/DR, CD20, FMC7, CD5, and CD9 antigens, had a significant influence on survival. On the other hand, several clinical and haematological characteristics were identified as being associated with survival: 1) clinical features--presence of lymphadenopathies (P less than .05) and hepatomegaly and/or splenomegaly (P less than .04); 2) haematologic parameters--presence of anaemia and/or thrombopenia (P less than .05), the absolute peripheral blood lymphocyte count (P less than .03), and the presence of hypogammaglobulinemia (P less than .08); 3) biochemical parameters--serum uric acid (P less than .03); and 4) bone marrow histopathological features--biopsy pattern (P less than .04) and the percentage of lymphocytes in bone marrow aspirate (P less than .03). Both the Rai staging and the International Workshop on CLL staging systems were effective in identifying groups of patients with significantly different prognoses (P less than .05). Multivariate regression analysis demonstrated that the combination of three clinicopathologic characteristics (bone marrow histopathologic pattern, absolute peripheral blood lymphocyte count, and the presence or not of hypogammaglobulinaemia) had the strongest predictive relationship with survival time. In summary, our findings show that the clinicobiological and anatomopathologic parameters have much more prognostic relevance than the immunological markers analysed in the present study.
 ...
PMID:B-cell chronic lymphocytic leukaemia: prognostic value of the immunophenotype and the clinico-haematological features. 270 40


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>