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)

Thrombocytopenia is a condition of unusually low level of platelets in blood, resulting from an imbalance between the production and destruction of platelets, and is associated with aplastic anemia, myelodysplasia, and idiopathic thrombocytopenic purpura (ITP). Thrombocytopenia can also be associated with severe chronic liver disease as a result of several factors that may act in concert, including reduced production of the endogenous thrombopoietic growth factor, thrombopoietin (TPO). This article examines the nature of thrombocytopenia, ITP, and TPO.
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PMID:Thrombopoietin agonists for the treatment of thrombocytopenia in liver disease and hepatitis C. 1962 64

Reticulated platelet (RP) is thought to be a useful marker for differential diagnosis and analysis of platelet kinetics in patients with thrombocytopenia. In this study, we compared two methods for the measurement of RP: flow cytometric (FCM) method and immature platelet fraction (IPF) method using automated hematology analyzer (XE-2000). There was a relatively good correlation between RP% measured by FCM method and IPF% measured by IPF method in patients with idiopathic thrombocytopenic purpura (ITP) (Y = 0.806X-0.050, r = 0.634, p < 0.001). We then measured RP% and IPF% in 61 patients with ITP and 27 patients with aplastic anemia (AA). For the differential diagnosis for ITP, the sensitivity (82%) and specificity (93%) of FCM method were better than those of IPF method (sensitivity 67% and specificity 63%). Our data demonstrate the significant difference between two methods by analyzing clinical samples in parallel.
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PMID:[Comparison between two methods for the measurement reticulated platelet and their clinical significance--flow cytometry (FCM) method and IPF method Using automated hematology analyzer (XE-2000)]. 2003 Jan 71

We measured plasma levels of thrombopoietin (TPO) in several patients with thrombocytopenia. Similar to previous reports, TPO levels in aplastic anemia (N=9) were markedly higher than those in idiopathic thrombocytopenic purpura (N=10): 16.19+/-9.07 fmol/ml and 1.21+/-1.06 fmol/ml, respectively. In patients with secondary failure of platelet recovery (N=7) as well as primary failure after hematopoietic stem cell transplantation, TPO levels were very high, reflecting impaired platelet production due to GVHD, drug treatments, and infection. When using new drugs such as TPO-receptor agonists, measurement of TPO levels might be important to differentiate the mechanism of thrombocytopenia.
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PMID:[Analyses of plasma thrombopoietin levels in patients with thrombocytopenia]. 2279 Jun 40

The natural hypoxic hypoxia enhances the synthesis processes and decrease the concentration balance in cytokine rets in patients with depression of hematopoiesis. It is established that the patients with aplastic anemia and idiopathic thrombocytopenic purpura, in contrast with patients with acute leucosis, chronic myelolecosis and erythroid myeloma, have quite high values of IL-2, which can be involved in the organization of full-fledgee response to antigen stimuli. The application of hypoxic hypoxia as an additional method in treatment of aplastic anemia and idiopathic thrombocytopenic purpura has no side effects and contraindications.
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PMID:[The dynamic detection of cytokins under aplastic anemia and idiopathic thrombocytopenic purpura in the process of impact of hypoxic hypoxia]. 2283 58

Aplastic anemia is characterized by a reduced hematopoietic stem cell number. Although GATA-2 expression was reported to be decreased in CD34-positive cells in aplastic anemia, many questions remain regarding the intrinsic characteristics of hematopoietic stem cells in this disease. In this study, we identified HOXB4 as a downstream target of GATA-2 based on expression profiling with human cord blood-derived CD34-positive cells infected with control or GATA-2 lentiviral shRNA. To confirm the functional link between GATA-2 and HOXB4, we conducted GATA-2 gain-of-function and loss-of-function experiments, and HOXB4 promoter analysis, including luciferase assay, in vitro DNA binding analysis and quantitative ChIP analysis, using K562 and CD34-positive cells. The analyses suggested that GATA-2 directly regulates HOXB4 expression through the GATA sequence in the promoter region. Furthermore, we assessed GATA-2 and HOXB4 expression in CD34-positive cells from patients with aplastic anemia (n = 10) and idiopathic thrombocytopenic purpura (n = 13), and demonstrated that the expression levels of HOXB4 and GATA-2 were correlated in these populations (r = 0.6573, p<0.01). Our results suggested that GATA-2 directly regulates HOXB4 expression in hematopoietic stem cells, which may play an important role in the development and/or progression of aplastic anemia.
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PMID:Gene expression profiling identifies HOXB4 as a direct downstream target of GATA-2 in human CD34+ hematopoietic cells. 2302 22

Conditions of hypoxic hypoxia at 3200 m height exert significant positive changes in hemopoiesis, normalizing erythropoiesis and coagulation system. Hypoxic climate therapy can be regarded as an additional efficient method to the pathogenetic treatment for patient with unpainful aplastic anemia and idiopathic thrombocytopenic purpura. It should be emphasized that patients must be out of immunosuppressive therapy when getting high altitude stationary.
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PMID:[Particular features of erythropoiesis in high altitude and possibilities of applying of hypoxic hypoxia methodology for the patients with hemopoietic suppression]. 2434 Oct 9

Parvovirus B19 is the causative agent of various forms of hematologic diseases such as aplastic crisis in patients with hemolytic anemia, aplastic anemia, hypoplastic anemia, and idiopathic thrombocytopenic purpura. In addition, parvovirus B19 infection may precede or be associated with acute lymphoblastic leukemia (ALL). The authors present two cases of parvovirus B19 infection and bone marrow infiltration with pre-B-cell lymphoblasts; one patients was diagnosed as having ALL, and the other patient, with neurologic findings, showed total resolution of the blastic morphology and phenotype.
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PMID:Parvovirus B19 Infection Presenting as Pre-B-Cell Acute Lymphoblastic Leukemia: A Transient and Progressive Course in Two Children. 2781 15

OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation. RESULTS Five patients with a bleeding diathesis who underwent surgery for intracranial hematoma were identified; the diagnosis was hemophilia A in 3 cases, idiopathic thrombocytopenic purpura in 1 case, and severe aplastic anemia in 1 case. Intracerebral hematoma (ICH) (n = 4) and acute subdural hematoma (n = 1) were confirmed on radiological investigations. In 2 of the 4 patients with ICH, the diagnosis of bleeding diathesis was made for the first time on presentation. Four patients (all male) were younger than 2 years; the patient with severe aplastic anemia and spontaneous ICH was 15 years old and female. The duration of symptoms varied from 24 hours to 5 days. Neurological examination at 1 year's follow-up showed complete recovery (GOS-E Peds score of 1) in 3 cases and mild weakness (GOS-E Peds score of 2) in 2 cases. CONCLUSIONS Neurosurgical management of patients with bleeding diathesis should be carried out in a tertiary-care setting with multidisciplinary team management, including members with expertise in neuroimaging and hematology, in addition to neurosurgery. Early diagnosis and prompt treatment of a bleeding diathesis is crucial for full neurological recovery.
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PMID:Neurosurgical management in children with bleeding diathesis: auditing neurological outcome. 2912 43

Eltrombopag is currently the only US Food and Drug Administration-approved thrombopoietin receptor agonist for the treatment of chronic immune thrombocytopenia (ITP) in children. This oral, once-per-day therapy has shown favorable efficacy and adverse effect profiles in children. Two multicenter, double-blind, placebo controlled clinical trials (PETIT [Efficacy and Safety Study of Eltrombopag in Pediatric Patients With Thrombocytopenia From Chronic Idiopathic Thrombocytopenic Purpura (ITP)] and PETIT2 [Study of a New Medication for Childhood Chronic Immune Thrombocytopenia (ITP), a Blood Disorder of Low Platelet Counts That Can Lead to Bruising Easily, Bleeding Gums, and/or Bleeding Inside the Body]) demonstrated efficacy in raising platelet counts, reducing bleeding, and reducing the need for concomitant ITP therapies with relatively few adverse effects. The most commonly reported drug-related adverse effects include headache, nausea, and hepatobiliary laboratory abnormalities. Long-term safety data in children are limited, and studies in adults have not revealed a clinically significant increased incidence of thrombosis, marrow fibrosis, or cataract formation. Eltrombopag has also been approved for treating refractory severe aplastic anemia (AA) and has potential for expanded use in ITP and severe AA as well as in other conditions associated with thrombocytopenia.
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PMID:Eltrombopag for use in children with immune thrombocytopenia. 2948 60


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