细胞学与组织学杂志

Thrombotic Microangiopathy and Low ADAMTS 13: Case Report and Review of the Literature

Abstract

Zakaria Almuwaqqat, Saad Ali, Abduljalil Elfasi, Harvey Freidman and Afsin Oktay

Background: Thrombotic Microangiopathy (TMA) is a multi-organ dysfunction syndrome characterized by microvascular thrombosis, thrombocytopenia and hemolytic anemia with diverse etiologies ranging from primary Thrombotic thrombocytopenic purura (TTP) and Hemolytic uremic syndrome (HUS) to secondary phenomena to medications, severe uncontrolled hypertension and autoimmune disorders i.e Scleroderma renal crises, catastrophic antiphospholipid syndrome and pre-eclampsia, prompt diagnosis, identification of potential etiologies and treatment are essential steps for reducing morbidity and mortality.
Case Report: We describe a case of a 34-year-old man who presented with features of advanced renal impairment, severe uncontrolled hypertension and thrombotic microangiopathy, overall clinical picture was most consistent with malignant hypertension and hypertensive nephrosclerosis secondary to long standing uncontrolled hypertension, however a serum test ordered measuring the activity of ADAMTS 13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) resulted one week later was consistent with reduced enzyme activity, challenging the first diagnosis.
Conclusions: Clinical correlation and high index of suspicion are essential in the diagnosis and management of thrombotic microangiopathy, The reduction in ADAMTS -13 activity or antigen level has been thought to be a primary cause of thrombotic microangiopathies I.e TTP and HUS, however recent studies have shown a negative correlation between ADAMTS-13 activity and elevated blood pressure (BP) in malignant hypertension.