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Acute respiratory distress syndrome and non-occlusive mesenteric ischemia as major clinical manifestations of thrombotic thrombocytopenic purpura: Complete remission following exchange plasmapheresis

Abstract

The typical manifestation of thrombotic thrombocyto-penic purpura (TTP) is the pentad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neuro-logic abnormalities, fever, and renal failure. However, the essential features of TTP are MAHA and thrombo-cytopenia [1], and diffuse microthrombitic lesions in the small blood vessels of one or more organs characterize the pathology of the syndrome. The central nervous sys-tem and kidney have been the most common target or-gans and, thus, the pentad of TTP has been derived. However, microthrombotic manifestation can occur in other organs, including the liver, kidneys, ocular regions, pancreas, and skin, resulting in predominant dysfunction of the particular organs, such as HELLP syndrome [2], hemolytic-uremic syndrome [3], macular detachment, choriocapillaris occlusion and retinal detachment [4], pancreatitis [5], and gangrenous skin lesions [6].An unusual case of TTP presenting as acute respira-tory distress syndrome (ARDS) and later recurring as acute non-occlusive mesenteric ischemia (NOMI) is en-countered. The clinical features of the patient are pre-sented and possible pathogenesis is discussed.

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