Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.6 (thromboplastin)
 13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemostatic studies found in the patients with Thrombotic thrombocytopenic purpura were discussed. Most of them showed normal routine hemostatic studies; normal prothrombin time, activated partial thromboplastin time, plasma level of fibrinogen. Fibrin degradation products are more consistently abnormal, with approximately to 70% having slight elevations, but less than 25% of titers are greater than 25 micrograms/ml. Although some cases concerning of complication of disseminated intravascular coagulation in the patient with TTP are found in the literature, laboratory and clinical finding suggest that TTP and DIC are separate entities.
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PMID:[Changes in molecular markers in patients with thrombotic thrombocytopenic purpura]. 843 5

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) and disseminated intravascular coagulation (DIC) may have identical manifestations in adults. Because TTP-HUS is 90% fatal without plasma exchange, prompt diagnosis is essential. To test the hypothesis that routine laboratory assays can discriminate between the 2 entities, we retrospectively identified adult patients with TTP-HUS and matched each with 2 patients with DIC. Although the platelet count, prothrombin time (PT), and partial thromboplastin time were different (P < .05) between the 2 patient groups, after regression analysis, only PT and profound thrombocytopenia remained associated with TTP-HUS (P = .001 and P = .003, respectively). A platelet count of less than 20 x 10(3)/microL (20 x 10(9)/L) and a PT within 5 seconds of the upper limit of the reference interval had a specificity of 92% for TTP-HUS. Our data confirm that readily available laboratory assays in the proper clinical scenario can increase the likelihood of TTP-HUS over DIC.
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PMID:Platelet count and prothrombin time help distinguish thrombotic thrombocytopenic purpura-hemolytic uremic syndrome from disseminated intravascular coagulation in adults. 2015 85

Acquired inhibitors of coagulation cause a life-threatening disease. Clinically newly occurring hemorrhagic diathesis combined with prolonged activated partial thromboplastin time (aPTT) time is diagnostically indicative and can be confirmed by a positive plasma exchange test. For thrombotic thrombocytopenic purpura and hemolytic uremic syndrome (TTP-HUS) the diagnosis of Coombs negative hemolytic anemia together with thrombocytopenia should lead to the detection of fragmentocytes in peripheral blood smears. Hairy cell leukemia is a rare subgroup of chronic B-cell neoplasia with the clinical signs of pancytopenia and splenomegaly which characteristically stain positive for CD103. The gastrointestinal stromal tumor (GIST) has nothing in common with classical soft tissue sarcoma based on the activating mutation of the KIT or PDGFRA gene (positivity for CD117). In all of these disorders the correct diagnosis has a major influence on patient outcome. For the case of acquired inhibitors of coagulation immunosuppressive therapy and substitution of coagulation factors (e.g. recombinant factor VIIa) or for TTP-HUS the immediate start of plasma exchange are mandatory. For hairy cell leukemia a very effective treatment exists with purine analogs (e.g. cladribine) and for metastatic inoperable GIST with tyrosine kinase inhibitors (e.g. imatinib).
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PMID:[Hemato-oncological diseases]. 2398 1