Therapeutic plasma exchange as part of multimodal treatment of acquired hemophilia in a patient with concurrent acute intracerebral bleed and pulmonary embolism. Transfusion 2017 Jul;57(7):1827-1832
Date
04/25/2017Pubmed ID
28436106DOI
10.1111/trf.14132Scopus ID
2-s2.0-85018921107 (requires institutional sign-in at Scopus site) 2 CitationsAbstract
BACKGROUND: Autoantibodies against Factor VIII (FVIII) define the rare but life-threatening bleeding disorder acquired hemophilia A (AHA). Correction of FVIII deficiency and eradication of the factor inhibitor are the ultimate therapeutic goals in this disorder. Bypassing agents such as recombinant factor VIIa (rFVIIa) or FVIII inhibitor bypassing agent are often used to control coagulopathy before the inhibitor is eradicated. Bypassing agents carry a risk of thrombosis, however.
CASE REPORT: We report a patient with newly diagnosed AHA and thalamic bleed who additionally had active atrial fibrillation and developed a segmental pulmonary embolism, limiting tolerable rFVIIa dosage. This patient with very-high-risk brain bleed and concurrent thrombosis on bypass agent represented a significant management dilemma for which we successfully utilized therapeutic plasma exchange (TPE) to reduce the inhibitor titer.
RESULTS: FVIII inhibitor was undetectable and FVIII level was above the lower limit of normal within 12.5 days from starting TPE. While the patient ultimately died 24 days from admission for reasons unrelated to bleeding, his intracerebral hemorrhage was unchanged in size and no other bleeding morbidity was observed.
CONCLUSION: This patient achieved eradication of FVIII inhibitor and did not have bleed expansion while receiving multimodal therapy including corticosteroids, rituximab, and TPE. We discuss the periprocedural risks of TPE in an acquired hemophilia patient and our multiteam management of that risk.
Author List
Wool GD, Chapel D, Treml A, Miller JLAuthor
Angela Treml MD Associate Professor in the Pathology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Acute DiseaseAged
Cerebral Hemorrhage
Factor VIII
Hemophilia A
Humans
Male
Plasma Exchange
Pulmonary Embolism
