How we approach an acquired thrombotic thrombocytopenic purpura patient.

Abstract

Acquired thrombotic thrombocytopenic purpura (TTP) is a disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, in addition to variable fever and neurologic and renal dysfunction, without an underlying cause. Recent advances in elucidating the pathophysiology of acquired TTP have led to new testing that we have incorporated into our current management of patients with suspected acquired TTP. Despite these developments, much of the treatment for acquired TTP beyond therapeutic plasma exchange (TPE) is based on low-quality evidence. Our group has a sustained interest in studying and optimizing the use of TPE, along with other concurrent therapies, in acquired TTP patients. Described herein is a summary of how our apheresis consult service approaches requests for TPE in patients with suspected acquired TTP.

• Authors: Jay S Raval, Marshall A Mazepa, Mark E Brecher, Yara A Park
• Journal: Transfusion (Transfusion) Vol. 54 Issue 10 Pg. 2375-82 (Oct 2014) ISSN: 1537-2995 [Electronic] United States
• PMID: 25070750 (Publication Type: Journal Article)
• Copyright: © 2014 AABB.

Chemical References

• ADAM Proteins
• ADAMTS13 Protein
• ADAMTS13 protein, human

Topics

• ADAM Proteins (genetics)
• ADAMTS13 Protein
• Algorithms
• Blood Component Removal (methods)
• Combined Modality Therapy
• Genetic Testing
• Humans
• Plasma Exchange (methods)
• Purpura, Thrombotic Thrombocytopenic (diagnosis, therapy)
• Referral and Consultation