Anti-ADAMTS13 (aa 1328-1427) polyclonal antibody

Thrombotic thrombocytopenic purpura (TTP) is a disease characterized by the presence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, caused by the congenital or acquired decrease of the enzyme activity which degrades unusual large vWF multimers. There is no identifiable cause in half of the acquired TTP cases. Herein, we report four possible pesticide-related cases with decreased ADAMTS13 enzyme activity, increased titer of ADAMTS13 inhibitor and typical clinical and laboratory presentation.

In India, an unexplained enteropathy is present in a majority of non-cirrhotic intrahepatic portal hypertension (NCIPH) patients. Small intestinal bacterial contamination and tropical enteropathy could trigger inflammatory stimuli and activate the endothelium in the portal venous system. Groundwater contaminated with arsenic is an environmental factor of epidemic proportions in large areas of India which has similar consequences. Von Willebrand factor (a sticky protein) expressed by activated endothelium may promote formation of platelet microthrombi and occlusion of intrahepatic portal vein branches leading to NCIPH. Environmental factors linked to suboptimal hygiene and sanitation, which enter through the gastrointestinal (GI) tract, predispose to platelet plugging onto activated endothelium in portal microcirculation. Thus, NCIPH, an example of poverty linked thrombophilia, is a disease mainly affecting the lower socio-economic strata of Indian population. Public health measures to improve sanitation, provide clean drinking water and eliminate arsenic contamination of drinking water are urgently needed. Till such time as these environmental factors are addressed, NCIPH is likely to remain 'an Indian disease'.