Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired thrombotic thrombocytopenic purpura (TTP), characterized by widespread thrombus formation in the microcirculation, is a ponderous complication of antiphospholipid syndrome. Recently, von Willebrand factor-cleaving protease (VWF-CPase) activity has been reported as a possible determinant for the occurrence of TTP. To clarify the role of VWF-CPase in the thrombus formation associated with antiphospholipid syndrome, we investigated plasma VWF-CPase activity in patients of collagen diseases with lupus anticoagulant (LA). Decreased plasma VWF-CPase activity less than 50% of the normal activity was observed in 25.7% (n = 18) in 70 patients with collagen diseases and 7 (10%) cases of them showed more lower VWF-CPase activity less than 25%. The IgG fractions obtained from 2 patients with the low VWF-CPase activity strongly inhibited the proteolytic reaction of normal VWF-CPase. There was no significant relationship between LA and plasma VWF-CPase activity. Thrombotic episodes, especially arterial thrombosis, were more frequently observed in LA-positive patients with low VWF-CPase activity. These results suggest that decreased activity of VWF-CPase, partly due to IgG type inhibitor to the enzyme activity may be an additional risk factor for arterial thrombosis in collagen disease patients with antiphospholipid antibodies.
 ...
PMID:[Von Willebrand factor-cleaving protease activity in patients of collagen disease with antiphospholipid antibodies]. 1198 61

In order to enhance the understanding of thrombotic thrombocytopenic purpura (TTP), the clinical features, laboratory characteristics, treatment and outcome of 14 patients with TTP were retrospectively analyzed and investigated. The results showed that 7 out of 14 patients with TTP had predisposing factors, such as pregnancy in 4 cases, infection in 3 cases, systemic lupus erythematosus (SLE) in 1 case and hematopoietic stem cell transplantation (HSCT) in 1 case. Fourteen patients all had neuropsychological symptoms, hemolytic anemia with negative-Coombs test, and decreased platelet counts. Eight patients had irregular fever with different degree. There were 8 patients with kidney damage including proteinuria in 8 cases and renal function abnormalities in 4 cases. The von Willebrand factor-cleaving protease (VWF-CP, ADAMTS13) activity of 13 cases out of 14 patients significantly decreased (less than 10%). At same time, plasma ADAMTS13 inhibitors were detected in 12 cases out of these 13 patients with decreased ADAMTS13 activity. After treatment with plasma exchange, glucocorticoid and rituximab so on, 12 cases achieved complete remission, in which 8 cases relapsed in two years. Two patients died at last, in which one case was secondary to HSCT. It is concluded that TTP is a kind of thrombotic microangiopathy due to platelet microthrombosis involved in multiple systems and multiple organs dysfunction with dangerous clinical process. The mortality of TTP patients is very high. Early diagnosis and early treatment with plasma exchange as the main means can greatly improve the prognosis of patients with TTP.
...
PMID:[Clinical analysis of 14 patients with thrombotic thrombocytopenic purpura]. 2476 14