Clinical Reviews
Thrombotic Thrombocytopenic Purpura: A Hematological Emergency

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Abstract

Background

Thrombotic thrombocytopenic purpura is a hematological emergency and diagnostic challenge. The critical determinant of outcome is timely diagnosis and treatment.

Objectives

Describe the pathophysiology, presentation, diagnosis, and treatment of thrombotic thrombocytopenic purpura.

Discussion

Thrombotic thrombocytopenic purpura has a varied presentation and a tendency to mimic several disorders. However, it may be at least provisionally diagnosed in the patient with thrombocytopenia and microangiopathic hemolytic anemia without alternate cause. The mainstay of treatment is immediate plasma exchange to be repeated until platelet count is stabilized. Adjuvant therapies include corticosteroids, rituximab, and cyclosporine.

Conclusion

It is essential for the emergency physician to be aware of thrombotic thrombocytopenic purpura’s range of presentations, diagnostic criteria, and treatment.

Introduction

Thrombotic thrombocytopenic purpura (TTP) is a disorder of platelet aggregation resulting in hemolysis of red blood cells, consumption of platelets, and occlusion of microvasculature. TTP was first described in 1925, and its classic pentad of features—fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, and renal impairment—was noted in 1966 (1). Until its effective treatment became defined in 1991, 90% of patients with TTP ultimately died 1, 2.

Today, it is known that the classic pentad is not typically present, and the mortality rate of TTP is still approximately 20% 2, 3. Deaths are often a result of delays in diagnosis and implementation of the appropriate therapy, rendering TTP a hematological emergency (4). Given the range and variability of presenting symptoms, and its tendency to mimic more common disorders, outcome hinges on the emergency physician’s suspicion for and knowledge of this rare but rapidly fatal disease.

Section snippets

Epidemiology

The most recent analysis of the incidence of TTP was done in 2005, and found an annual incidence rate of patients treated for suspected TTP to be about 11 cases per 1,000,000 persons. This estimate is notably higher than previous ones based on death certificates and insurance claims. The same study noted that the incidence rate of TTP is greater in women than men, in Blacks than non-Blacks, and those living in urban settings than rural areas. The former two findings are consistent with the

Conclusion

TTP is a rare but true hematological emergency with fatal consequences if not promptly diagnosed and appropriately treated. Its presentation is variable with non-specific symptoms and imitates common disease processes, often leading clinicians to erroneous diagnoses and causing unaffordable delays. Thus, it is imperative for the emergency physician to be familiar with diagnostic and therapeutic guidelines.

TTP classically presents with fever, thrombocytopenia, microangiopathic hemolytic anemia,

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