Thrombotic thrombocytopenic purpura: the challenges of a complex disease process

Thrombotic thrombocytopenic purpura is an acute, rare disorder with a poor prognosis, though the survival rate has improved dramatically in the past 20 years. It is a complex disease characterized by thrombocytopenia, hemolytic anemia, fluctuating neurologic symptoms, fever, and renal dysfunction. The incidence of thrombotic thrombocytopenic purpura appears to be increasing in frequency, with women affected twice as often as men. The median age of onset is in the fourth decade of life. The clinical manifestations of thrombotic thrombocytopenic purpura are caused by widespread deposition of platelet microthrombi that occlude the capillaries and arterioles, especially evident in the brain, kidney, and bone marrow. The resulting intravascular platelet consumption is responsible for bleeding and petechiae. The etiology is unknown, but may be caused by immune system mediated damage to the endothelium. The complexity of this disease challenges the health care team and demands highly skilled medical and nursing management. This article presents a summary of the history and pathophysiology of the disease, clinical course with case study, medical treatment, nursing diagnoses and interventions, and conclusions.