Thrombotic Thrombocytopenic Purpura: Etiopathogenesis, Diagnostics and Basic Principles of Treatment

Abstract

Th rombotic thrombocytopenic purpura (TTP) is a clinical syndrome that manifests with thrombocytopenia, microangiopathic haemolytic anaemia and symptoms and signs of kidney and brain damage, but it rarely involves other organs. Th e main pathophysiological cause of TTP is diminished metalloproteinase ADAMTS13 activity; the main function of ADAMTS13 is to degrade large multimers of the von SAŽETAK Trombotična trombocitopenijska purpura (TTP) je klinički sindrom koji se odlikuje trombocitopenijom, mikroangiopatskom hemoliznom anemijom i simptomima i znacima oštećenja bubrega i mozga, ređe drugih organa. Glavni patofi ziološki mehanizam nastanka TTP-a je smanjena aktivnost metaloproteinaze ADAMTS13 čija je osnovna uloga razgradnja velikih multimera von Willebrand-ovog faktora. Smanjena aktivnost metaloproteinaze ADAMTS13 nastaje usled mutacije u genu za ADAMTS13 (urođeni TTP) ili usled nastanka antitela koja blokiraju ili antitela koja ubrzavaju razgradnju ADAMTS13 (stečeni TTP

Acute Kidney Damage in Pregnancy: Etiopathogenesis, Diagnostics and Basic Principles of Treatment

“…Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome that is characterized by thrombocytopenia, microangiopathic hemolytic anemia, high body temperature, disorders of the central nervous system and acute renal insuficiency (28)(29)(30). It is caused by decreased activity of ADAMTS13 metalloproteinase, an enzyme that ctears apart macromolecules ofvon Wilebrand's factor (vWF) secreted by endothelial cells of small blood vessels, and this results in the formation of clots (vWF + platelets), and the narrowing of lumen of the small blood vessels of the brain and kidney (28)(29)(30). Depending on the causes leading to reduced ADAMTS13 activity, we distinguish congenital (mutation of gene for ADAMTS13 synthesis) and acquired (anti-ADAMTS13 IgG antibodies) form of TTP (28)(29)(30).…”

“…It is caused by decreased activity of ADAMTS13 metalloproteinase, an enzyme that ctears apart macromolecules ofvon Wilebrand's factor (vWF) secreted by endothelial cells of small blood vessels, and this results in the formation of clots (vWF + platelets), and the narrowing of lumen of the small blood vessels of the brain and kidney (28)(29)(30). Depending on the causes leading to reduced ADAMTS13 activity, we distinguish congenital (mutation of gene for ADAMTS13 synthesis) and acquired (anti-ADAMTS13 IgG antibodies) form of TTP (28)(29)(30). In congenital TTP (cTTP), ADAMTS13 activity is ≤ 5%, and for aquired TTP (sTTP) ≤ 10% (28)(29)(30).…”

“…Depending on the causes leading to reduced ADAMTS13 activity, we distinguish congenital (mutation of gene for ADAMTS13 synthesis) and acquired (anti-ADAMTS13 IgG antibodies) form of TTP (28)(29)(30). In congenital TTP (cTTP), ADAMTS13 activity is ≤ 5%, and for aquired TTP (sTTP) ≤ 10% (28)(29)(30). The incidence of congenital thrombotic thrombocytopenic purpura in pregnancy is 1/200,000 pregnancies.…”

“…The incidence of congenital thrombotic thrombocytopenic purpura in pregnancy is 1/200,000 pregnancies. In pregnant women, thrombotic thrombocytopenic purpura is clinically represented by headache, confusion, visual impairment, proteinuria, pre-eclampsia, kidney damage, abdominal pain, embolization of the lungs and acute respiratory distress syndrome (28)(29)(30). The congenital form of TTP associated with pregnancy most commonly occurs in the postpartum period (monitoring for cTTP in the postpartum period).…”

“…The congenital form of TTP associated with pregnancy most commonly occurs in the postpartum period (monitoring for cTTP in the postpartum period). Treatment begins with plasmapheresis (plasmapheresis is the first line of therapy) (28)(29)(30). It is applied on a daily basis for five days, at least one plasma volume (2800 ml) is changed, and fresh plasma is used to substitute the separated plasma.…”