Coagulation abnormalities in type 1 Gaucher disease are due to low-grade activation and can be partly restored by enzyme supplementation therapy
Autor: | Johannes M. F. G. Aerts, F. Berends, Marcel Levi, C.E.M. Hollak, M. H. J. Van Oers |
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Přispěvatelé: | Faculteit der Geneeskunde |
Rok vydání: | 1997 |
Předmět: |
Adult
Male medicine.medical_specialty medicine.medical_treatment Enzyme-Linked Immunosorbent Assay Gastroenterology Alglucerase Internal medicine Fibrinolysis medicine Humans Platelet Coagulation Disorder Aged Clotting factor Prothrombin time Gaucher Disease biology medicine.diagnostic_test Platelet Count business.industry Factor V Blood Proteins Hematology Blood Coagulation Disorders Middle Aged Blood Coagulation Factors Immunology Prothrombin Time Splenectomy biology.protein Glucosylceramidase Female Partial Thromboplastin Time business Partial thromboplastin time medicine.drug |
Zdroj: | ResearcherID British journal of haematology, 96, 470-476. Wiley-Blackwell |
ISSN: | 0007-1048 |
Popis: | In type 1 Gaucher disease a bleeding tendency occurs which is partly caused by thrombocytopenia due to massive splenomegaly. In addition, low levels of factors IX and XI have been described. The mechanism responsible for these clotting factor abnormalities is unknown. We performed a detailed study of parameters of coagulation and fibrinolysis in 30 type 1 Gaucher disease patients (14 splenectomized) before and after treatment with enzyme supplementation therapy. Pre-treatment aPTT and PT were prolonged in 42% and 38% of patients, respectively. In 30-60% serious deficiencies (< 50%) of coagulation factors XI, XII, VII, X, V and II were observed. The low levels of factor V correlated with platelet count and were inversely associated with splenic volume. Levels of inhibitors were mildly decreased. Markers for activation of coagulation (thrombin-antithrombin (TAT) complex) and fibrinolysis (PAP complex, fibrin cleavage product D-dimer) were significantly elevated, especially in the splenectomized patients, indicating ongoing activation of these processes. After 12 months of enzyme supplementation therapy partial correction occurred. Thus, severe disorders of the coagulation system occur in Gaucher disease, contributing to the bleeding tendency. The deficiencies may be the result of consumption of coagulation factors caused by ongoing low-level coagulation activation. possibly due to mononuclear cell activation. |
Databáze: | OpenAIRE |
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