Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

By means of a bioassay performed in rats utilizing 75Se-selenomethionine as a platelet marker in vivo, serum thrombocytopoietic activity (STA) was determined in 9 healthy volunteers and in 82 patients with thrombocytopenia, viz. 27 cases of ITP (9 acute and 18 chronic), 19 of aplastic anaemia, 19 of acute leukaemia and 17 of chronic proliferative disorders. STA was the highest in the ITP group (p less than 0.01), particularly in the cases of short duration (p less than 0.001). In acute leukaemia, the STA was remarkably elevated (p less than 0.02) and a negative correlation between STA and blood platelet count could be demonstrated. A negative correlation between STA and the duration of thrombocytopenia was also found in the patients with ITP. No significant increase of STA was observed in patients with ITP after corticosteroid administration. In 5 out of 7 cases who responded satisfactorily to splenectomy a significant fall of STA was demonstrated after surgery. A slight, but statistically significant, rise of the STA value was found in patients with aplastic anaemia (p less than 0.05). No STA elevation was observed in the cases of chronic proliferative disorders with thrombocytopenia.
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PMID:Serum thrombocytopoietic activity in thrombocytopenias. 57 41

Sera from 23 patients with idiopathic thrombocytopenic purpura (ITP), 14 patients with aplastic anemia with severe thrombocytopenia and healthy control subjects were tested for the presence of fibrinogen/fibrin degradation products (FDP), using the tanned red cell hemagglutination inhibition immunoaassay. The concentrations of circulating FDP of ITP patients (mean 12.01 mug/ml) were significantly higher than those of the patients with aplastic anemia (mean 4.01 mug/ml, p less than 0.05) or normal controls (mean 3.10 mug/ml, p less than 0.001). The patients with untreated ITP with very low platelet counts had higher levels of FDP than those of the treated group (p less than 0.01). Serum FDP and a battery of other coagulation-fibrinolysis tests were serially carried out over a period of 10 weeks in two patients with ITP. The initially high FDP promptly decreased as circulating platelets increased in response to steroid in both patients, while plasma fibrinogen, euglobulin lysis time, prothrombin time and partial thromboplastin time remained essentially normal during the course of observation. The exact source of the increased serum FDP in ITP was not established, but a few possible mechanisms responsible for this abnormality were discussed.
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PMID:Fibrinogen/fibrin degradation products in serum of patients with idiopathic thrombocytopenic purpura: elevated levels during severe thrombocytopenic phase of the disease. 98 69

Reactive changes in the lymphoid cells are seen in patients with active rheumatoid arthritis and the degree of reactivity is related to the activity of the disease. The test may be useful as an early indication of relapse in autoimmune disease. Changes are also described in the lymphoid cells of the blood in patients with idiopathic thrombocytopenic purpura, which are identical with those seen in other autoimmune disorders, in infections, and following immunization. This provides supporting evidence for the autoimmune nature of this disease. In contrast with this, patients with aplastic anaemia have been found to have entirely normal lymphoid cell populations in the peripheral blood and provide no evidence that this condition has an autoimmune basis.
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PMID:Reactive lymphoid cells ('Immunoblasts') in autoimmune and haematological disorders. 124 46

Corticosteroids were used to treat two children with presumed idiopathic thrombocytopenic purpura and one with juvenile rheumatoid arthritis without examination of the bone marrow. Of the two with presumed idiopathic thrombocytopenic purpura, one had Fanconi's anaemia and the other may have had aplastic anaemia. The third child had acute lymphoblastic leukaemia. The diagnosis of Fanconi's anaemia was delayed. A diagnostic and therapeutic dilemma was caused in the second case. In the third, delayed diagnosis and, perhaps, compromised outlook resulted. These three cases re-emphasize the well aired caveats about the diagnosis of idiopathic thrombocytopenic purpura and juvenile rheumatoid arthritis and provide further support for the arguments of those who believe that if corticosteroids are to be used to treat such children, their bone marrow should be examined first.
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PMID:Bone marrow examination before steroids in thrombocytopenic purpura or arthritis. 129 Aug 52

We determined platelet-associated IgG (PAIgG) levels in patients with aplastic anemia, idiopathic thrombocytopenic purpura (ITP), iron deficiency anemia, and systemic lupus erythematosus (SLE), as well as in normal healthy adults as a control group. To determine PAIgG levels, we used a competitive micro enzyme-linked immunosorbent assay, which had excellent reproducibility, recovery, and dilution. We confirmed its reliability by comparing it to the immunoradiometric assay. Both the aplastic anemia group (n = 27, mean +/- SD = 218.6 +/- 244.6 ng/10(7) platelets) and the ITP group (n = 82, mean +/- SD = 212.5 +/- 327.8 ng/10(7) platelets) had higher PAIgG levels than the SLE group (n = 4, mean +/- SD = 38.4 +/- 22.4 ng/10(7) platelets), iron deficiency anemia group (n = 10, mean +/- SD = 16.1 +/- 3.6 ng/10(7) platelets), and normal control group (n = 69, mean +/- SD = 16.1 +/- 3.6 ng/10(7) platelets. The higher platelet-associated IgG levels in aplastic anemia suggest that autoimmune mechanisms are involved.
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PMID:Elevation of platelet-associated IgG in aplastic anemia. 150 79

Regulation of megakaryocyte and platelet production remains poorly understood. In culture system two separate activities are needed for maximum production of megakaryocyte progenitors: promotor of clonal expansion and promoter of maturation, other growth factors and cells also contribute to regulation of megakaryocytopoiesis. Increased proliferation of megakaryocytes is observed in myeloproliferative disorders and idiopathic thrombocytopenic purpura, and decreased proliferation is found in aplastic anaemia and hypomegakaryocytic thrombocytopenia. Dysmegakaryocytopoiesis is present in myelodysplastic syndromes and acute leukaemia, and a proliferation of immature megakaryocytes in acute megakaryoblastic leukaemia. Increased understanding of human megakaryocytopoiesis is beginning to help in rational clinical management.
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PMID:Human megakaryocytopoiesis--normal and abnormal. 169 25

Plasma Interleukin-6 (IL-6) level was measured in patients with idiopathic thrombocytopenic purpura (ITP), systemic lupus erythematosus (SLE), rheumatoid arthritis and aplastic anemia. Increase in the plasma level of IL-6 was observed in patients with ITP and SLE. The plasma IL-6 level decreased with progression of the treatment for ITP, and it showed weak negative correlations with the platelet count at the onset of ITP. The increases in the plasma IL-6 level suggest the involvement of activation of the immune system in the pathogenesis of ITP.
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PMID:[Elevated plasma interleukin-6 in patient with idiopathic thrombocytopenic purpura]. 192 Aug 40

Sialyl Lewisx-i (SLX) was found in more than 40% of patients with acute leukemia or chronic myelogenous leukemia, and in about 20% of those with myelodysplastic syndrome or malignant lymphoma. This tumor marker was absent in all patients with polycythemia vera, essential thrombocythemia, primary myelofibrosis, chronic lymphatic leukemia, multiple myeloma, and those with acute leukemia or malignant lymphoma in remission. The marker was found in 8% and of the patients with idiopathic thrombocytopenic purpura and 33% of those with autoimmune hemolytic anemia but in no patient with aplastic anemia or megaloblastic anemia. Immunostaining with SLX antibody showed that tumor cells of the patients with high levels of serum SLX were producing the SLX antigen. The detection of this marker in the serum is thought to be useful not only in the diagnosis but also in the observation of the recurrence of the diseases.
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PMID:Evaluation of serum sialyl Lewisx-i in hematologic disorders. 207 71

Therapies and prognoses covering fifteen cases of intracranial hematoma (ICrH) accompanying various types of bleeding tendency (BTD) were studied along with a secondary analysis of the pertinent references. Fifteen cases were divided into two groups, Group A comprising 11 cases of ICrH accompanying primary BTD, and Group B comprising four cases of ICrH accompanying secondary BTD caused by various underlying diseases. Group A included four cases of hemophilia A (Hp-A), two cases of factor XIII deficiency (FXIII-d), three cases of thrombocytopenia (Th-p) and two cases of vitamin K deficiency (VK-d). The four cases of Hp-A responded favorably, with good prognoses, to a supplementary therapy alone. This result was endorsed by the development of therapy as documented in the references. The combined five cases of FXIII-d and Th-p tended without exception, to show good prognoses in the wake of a combination therapy of supplementary treatment and surgical procedure. As regards FXIII-d, there was an inter-reference difference in supplementary doses. Many references shared the view that splenectomy was essential to the treatment of Th-p in general, and idiopathic thrombocytopenic purpura in particular. The current study also suggested that gammaglobulin in large doses would serve as an effective therapy. The two cases of VK-d suffered from a serious degree of lingering neurologic manifestations, although their lives were saved. Even though there is an established therapy for it, VK-d was found to be a problem with poor functional prognosis showing the importance of the preventive approach. Group B was classified into the acute type and the subacute type depending on the rate of pathologic development. As underlying diseases DIC and myelofibrosis due to acute myeloblastic leukemia, and Th-p due to aplastic anemia were noted in two cases in each group. Of these, two cases of the subacute type were able to be saved, while two cases of the acute type followed poor prognostic courses resulting, eventually, in death. The following were found to be responsible fatal factors: 1) causes of BTD which involved both mechanisms of coagulation and hemostasis, 2) non-removal of the underlying disease, in which case supplementary therapy tended to be futile, and 3) the underlying disease per se as a danger to the life of the patient. In conclusion, therapeutic rationale and prognosis in ICrH accompanying primary type of BTD will benefit from the implementation of an adequate augmentative therapy as in the ordinary type of ICrH.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Intracranial hematoma accompanying bleeding tendency: therapeutic practice and analysis of literature]. 220 82

We measured the calmodulin content in platelets in 13 normal persons and in 62 patients with hematological diseases. The level of platelet calmodulin was higher in patients with idiopathic thrombocytopenic purpura (ITP), systemic lupus erythematosus, myeloproliferative disorders, acute leukemia in a recovery phase, aplastic anemia, thrombosis and hypersplenism as compared to the controls. Among the patients with ITP, calmodulin was lower in responders than in nonresponders and those at the initial diagnosis. We also measured the volume, life-span and aggregation of the platelets and demonstrated a significant relationship between the calmodulin level and the platelet volume, and a negative relationship between the calmodulin level and platelet life-span, there was no correlation between the calmodulin level and platelet aggregation. We thus conclude that platelet calmodulin is inversely correlated with platelet turnover.
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PMID:Platelet calmodulin correlates with platelet turnover. 230 19


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