Abstract: PUB314
Recurrent Thrombotic Thrombocytopenic Purpura: A Case with an Aggressive Trigger Factor
Session Information
Category: Pathology and Lab Medicine
- 1800 Pathology and Lab Medicine
Authors
- Oyedeji, Akintunde, Meharry Medical College, Nashville, Tennessee, United States
- Awosanya, Tiwalade, Meharry Medical College, Nashville, Tennessee, United States
- Nzerue, Chike, Meharry Medical College, Nashville, Tennessee, United States
Introduction
TTP is caused by severely deficient activity of the ADAMTS13 protease which is responsible for cleaving large VWF molecules to smaller sized multimers which prevents abnormal accumulation and attachment of platelets to the endothelial surface. It is caused by autoantibodies against ADAMTS13. Infections like E. coli have been noted to be a trigger.
Case Description
We present a case of a 50-year-old African-American male who presented with a 1 week history of recurrent fever, diarrhea and nausea/vomiting,hematochezia and hematemesis.Platelet count:11,000 with hemoglobin of 6.6.GI panel was positive for Enteroaggressive E. coli. ADAMTS13 was found to be <5L. Antibodies to ADAMTS 13 were detected
He was started on solumedrol, TPE x6 and discharged on oral prednisone to be commenced on Rituxan. Platelet count had improved to 187,000 at discharge.
He had a relapse a year later presenting with similar symptoms. Platelet count:15. He had steroids, Rituxan and TPE x5 and was discharged with platelet count of 223. 9 months later he had a relapse and was admitted to another facility where he was managed for recurrent TTP c/b type 2 MI and required 3 TPE x3 with high dose steroids, caplacizumab, and 1/4 dose of rituximab. He self discontinued Caplacizumab due to severe lower back pain. He presented a week later to our facility with platelet count of 6. He received Rituximab and TPE x5 and was discharged with platelet count of 150,000.At the last visit his platelet count was 300,000 off all medications and he is currently doing well.
Discussion
TTP is a life-threatening disease that requires early recognition and treatment. TTP can be congenital or immune-mediated (acquired TTP). Acquired TTP is associated with anti–ADAMTS 13 protease antibodies and remains the main cause of TTP (70-80%) as was seen in this patient. Factors that can trigger TTP include infection, vaccinations, and certain medications.Prevention of relapses is crucial, and therefore identification of the trigger is critical.Initial treatment of an acute episode with Rituximab together with PEX and corticosteroid decreases length of hospitalization.
This case report highlights a rare inciting factor for recurrent TTP and the need for more studies to determine the etiology of relapses following the initial event.