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)

The combination of idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia is rare in childhood. Among 164 instances of ITP and 15 instances of AHA, 11 patients were found to have this combination. Three were found to have systemic lupus erythematosus, one had aplastic anemia, and seven had Evans syndrome. Neutropenia, at times associated with bacterial infections, occurred in four of the latter patients. Unlike most cases of ITP or AHA in childhood, the clinical course of Evans syndrome is usually chronic and relapsing. Treatment including corticosteroids, splenectomy, and immunosuppressive agents has been generally unsatisfactory. In view of the frequent presence of antibodies directed at red blood cells, platelets, neutrophils, and lymphocytes, immunopancytopenia may be a better term for this condition.
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PMID:Evans syndrome in childhood. 719 90

The presenting clinical pictures and courses of seven patients with thrombocytopenia, decreased megakaryocytes in the marrow, and minimal changes in other hematopoietic cell lines are described. Little information exists in the literature on such patients. Initial bone marrow aspiration and biopsy in all patients showed decreased megakaryocytes with an otherwise normal marrow. Erythrocyte mean corpuscular volume was elevated in five of seven patients. Bone marrow karyotypes of six of the seven patients were normal. Chromium-51 platelet survival studies with platelet sizing, done in five of the seven patients, showed normal results. In two patients the course progressed to aplastic anemia. One of these died 9 months after presentation, and one responded dramatically to lithium. One patient developed preleukemia and died. The other four patients have remained thrombocytopenic but clinically stable. No useful therapy was identified. The differential diagnosis of such patients should include idiopathic thrombocytopenic purpura with misinterpretation of morphologic findings, hereditary and acquired aplastic anemia, preleukemia, and systemic lupus erythematosus.
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PMID:Thrombocytopenia with decreased megakaryocytes. Evaluation and prognosis. 719 25

The International Committee for Standardization in Hematology recommended three methods for calculating objectively the mean platelet survival time, ie, weighted mean of linear estimate and logarithmic estimate, truncated exponential model, and gamma model. For determining which method is better, data of the survival study were fitted to each of three methods, as well as the Paulus polynomial and exponential polynomial models, and the mean survival time and the mean square error were compared. Mean survival obtained by each method was always longest in healthy controls and shortest in ITP. In 3rd degree of polynomial estimate and 2nd and 3rd degrees of logarithmic estimate, several cases showed practically outlying mean survival time of more than 12 days. In healthy controls, highest mean square error was observed in 1st degree logarithmic estimate, and in cases with short mean survival time the highest one was obtained in 1st degree polynomial estimate. Mean square error in ITP pre- and postsplenectomy and in aplastic anemia was smallest in the gamma model. Among nine methods recommended by the International Committee for Standardization in Hematology and by Dr. Paulus, the gamma model so far provides the best method for calculating the mean survival time of 51Cr-platelets.
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PMID:On the methods calculating mean survival time in 51Cr-platelet survival study. 741 59

Antiviral humoral immunity was studied in patients with Hodgkin's disease, idiopathic thrombocytopenic purpura (ITP), and aplastic anemia (AA). Insufficiency of antiviral humoral immunity as regards antigenically unrelated viruses and Mycoplasma pneumoniae was characteristic of all these conditions. A remission of Hodgkin's disease and ITP was associated with a reliable increase of the studied parameters in comparison with the acute period of the disease. After splenectomy humoral immunity parameters were virtually unchanged in patients with ITP, whereas in patients with Hodgkin's disease and AA the deficiency of antibody production is 40% increased after the operation.
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PMID:[Humoral antiviral immunity in children with hematologic neoplasms]. 748 71

A morphometric analysis was performed on aspirate clots of bone marrow to identify the presence of atypical megakaryocytes after immunohistological staining with a monoclonal antibody against Factor VIII. This study included cases of myelodysplastic syndrome (MDS), myeloproliferative disorder (MPD), aplastic anaemia (AA), idiopathic thrombocytopenic purpura (ITP), chronic myelogenous leukaemia (CML), and control cases free from any haematological disease. Quantitative and qualitative abnormalities of megakaryocytes were assessed using an image analyser and a personal computer to perform a morphometric analysis of the number (per mm2), arrangement (microns), nuclear size (microns2), cell size (microns2), nuclear size/cell size (N/C) ratio, and nuclear contour index (NCI). Micromononuclear megakaryocytes were detected in MDS, while in MPD, large over-mature magakaryocytes were observed to increase in number. In AA, the megakaryocytes decreased dramatically in number without showing any morphological abnormality. In CML, the megakaryocytes also increased without any remarkable morphological abnormality. In ITP, only the NCI increased. The above results show that MDS is characterized by the presence of atypical micromegakaryocytes, while MPD is characterized by atypical large over-mature megakaryocytes.
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PMID:A megakaryocyte analysis of the bone marrow in patients with myelodysplastic syndrome, myeloproliferative disorder and allied disorders. 749 Jun 85

We described a fully automated measurement of reticulated platelets using a fluorescent dye, auramine O, and a reticulocyte counter, the R-3000, equipped with special software. Reproducibility and linearity were shown to be good. In the normal subjects studied (n = 60), the mean value for reticulated platelets was 0.98% +/- 0.41% and the mean absolute count was 2.12 +/- 0.69 x 10(9)/l. The absolute count for reticulated platelets was significantly lower (p < 0.05) in patients with reduced thrombopoiesis as seen in acute myeloblastic leukemia, aplastic anemia or chemotherapy-induced thrombocytopenia and it was elevated (p < 0.05) in essential thrombocythemia and in chronic myelocytic leukemia with thrombocytosis. All 20 patients with chronic idiopathic thrombocytopenic purpura had a high percentage of reticulated platelets. The percentage of reticulated platelets was significantly increased (p < 0.05) in patients with impaired thrombopoiesis despite the reduction in the absolute count. In 2 leukemic patients, an apparent rise was noticed in the percentage of reticulated platelets which preceded by several days a progressive increase in the platelet count at the recovery phase of thrombocytopenia. The results suggest that an automated measurement of reticulated platelets can be applied to routine laboratories for clinical use.
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PMID:Automated measurement of reticulated platelets in estimating thrombopoiesis. 772 Aug 36

Development of the method to determine reticulated platelets is briefly reviewed. A new rapid method to automatically count reticulated platelets is very recently established by our research group. The principle of the measurement of reticulated platelets is based on flow cytometry. The platelets are quickly stained with a RNA fluorescent dye, auramine O and fluorescent intensity (RNA content) and forward scatter (cell size) are measured in only 80 seconds with a reticulocyte counter, equipped with special software for analysis of reticulated platelets. Both of the reproducibility and the linearity were shown to be good. Normal percentage value for reticulated platelets was 0.98% +/- 0.41% and its absolute count was 2.12 +/- 0.69 x 10(9)/l. The absolute count was decreased in patients with reduced thrombopoiesis such as acute myeloblastic leukemia, aplastic anemia and was elevated in patients with essential thrombocythemia and in chronic myelocytic leukemia. The patients with chronic idiopathic thrombocytopenic purpura had a high percentage of reticulated platelets. An apparent rise was noticed in the percentage of reticulated platelets which preceded by several days a progressive increase in the platelet count at the recovery phase of thrombocytopenia in a couple of leukemic patients. These suggest that an automated measurement of reticulated platelets can be clinically useful to estimate thrombopoiesis in bone marrow.
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PMID:[Reticulated platelets--automated measurement and clinical utility]. 778 28

Platelet phospholipid dependent thrombin generation was determined in 20 patients with thrombocytosis, 18 with thrombocytopenia, and 25 normal controls, using a quantitative chromogenic assay. Platelets from patients with myeloproliferative disorders displayed normal lag times to 20 nM thrombin concentration but increased thrombin potentials, even when corrected for platelet size. Platelets from patients with reactive thrombocytosis supported normal thrombin generation. ITP platelets were large, with a proportionate increase in thrombin potentials, but very short lag times to 20 nM thrombin concentration. Following marrow ablation there was a progressive loss of activation-induced enhancement of thrombin generation. Platelets from patients with idiopathic aplastic anaemia supported normal thrombin generation. These findings indicate that platelet phospholipid-dependent thrombin generation is altered in many patients with a variety of quantitative platelet disorders, and this may be an important determinant of the clinical expression of these disorders.
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PMID:Altered platelet phospholipid-dependent thrombin generation in thrombocytopenia and thrombocytosis. 783 51

Recently there have been increasing reports of HIV infection acquired through transfusion of HIV seronegative blood in Thailand due to high incidence of HIV new infection in blood donors. Blood or blood components (BC) prepared from HIV seronegative blood donation pose significant hazards to recipients because of the risk of viremia during the "window period" of HIV infection. This paper presents the HIV seroprevalence in hematologic patients other than hemophiliacs who received multiple blood transfusion at Ramathibodi Hospital. The retrospective analysis was done on 167 patients: 132 thalassemia, 19 leukemia, 5 aplastic anemia, 5 ITP, 2 pure red cell aplasia, 2 congenital non spherocytic hemolytic anemia, 1 hereditary spherocytosis and 1 autoimmune hemolytic anemia patients, who received blood transfusion during January 1, 1987 till February 29, 1992 at the Department of Pediatrics, Ramathibodi Hospital. The number of blood or BC transfused in each patient was 1-154 units with the average of 23 units per patient per 5 years with a total 4,000 units. All were HIV sero-negative. Anti-HIV screening was performed periodically in these patients about 1-2 times per year or as necessary. The results were HIV seronegative in all cases. The reason for negative results cannot be explained clearly. It should be noted that our thalassemic patients receive leukocyte poor blood and avoid a hypertransfusion program. Patients with other blood diseases received both whole blood and BC. The HIV contaminated blood in the window period was estimated to be 1:10,000 in Thailand which showed HIV antigen positive but antibody negative. These patients may be fortunately received HIV non contaminated blood.
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PMID:HIV seroprevalence in hematologic patients other than hemophiliacs at Ramathibodi Hospital. 788 70

To clarify the prognosis of patients with intractable diseases, a baseline survey of patients who received financial aid for intractable disease treatment between April 1984 and March 1985 was performed in Wakayama Prefecture, followed by a follow-up survey over a period of 8 years. Based on public welfare-subsidized system in Wakayama Prefecture, all patients with intractable diseases were checked up annually their certificates of financial aid for treatment. The results obtained were as follows: Parkinson's disease had the highest rate of discontinuation of medical care at 65%, followed by idiopathic thrombocytopenic purpura (ITP) at 64%, aplastic anemia at 55%, ulcerative colitis (UC) at 54%, and Behcet's disease at 50%. Systemic lupus erythematosus (SLE) had the lowest rate of discontinuation of medical care at 32%. Discontinuation was due to death in approximately 50% of the patients with SLE, scleroderma, dermatomyositis and polymyositis, and aplastic anemia. Of these, the causes of death in more than 50% were directly related to the primary diseases. However, in patients with SLE and aplastic anemia, 25% discontinued care because of cure or alleviation. Some patients remained in remission even though the prognosis for these diseases is not generally considered to be favorable. Transfer of jurisdiction to the Disabled Persons Welfare Act was seen in 28% of patients with Parkinson's disease, 24% with Behcet's disease, and 23% with ossification of posterior longitudinal ligament, diseases which are believed to cause restriction in daily activities of patients in some cases. On the other hand, cure or alleviation was the reason for discontinuation in 60-70% of patients with Buerger's disease, UC and ITP.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Prognosis of patient with intractable diseases in Wakayama Prefecture--a follow-up study based on medical care certificates]. 802 9


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