C52-28 A Refractory Case of Peripartum Thrombotic Thrombocytopenic Purpura (TTP)

Thrombotic Thrombocytopenic Purpura is a rare disorder resulting from a deficiency of ADAMTS13, a von Willebrand Factor (vWF) protease. vWF binds platelets for hemostasis. A lack of ADAMTS13 - often secondary to autoantibodies - leaves vWF as large multimers that excessively binds platelets, causing hemolytic anemia, thrombocytopenia, microvascular thrombi, and ischemia in the kidneys, brain, and heart. The mainstays of treatment include immune suppression via steroids, plasma exchange, and in severe cases - antibodies that inhibit plasma cells or block platelet-vWF binding. Ms. D is a 30 year old G4P1021 female who presented at 37W1D for worsening back pain, found to have category 3 fetal tracings and was taken for an emergent caesarian section. Prior to surgery, her hemoglobin was 5.2 (9.7 two months prior) with a platelet count of 13. She was initially thought to have HELLP versus hemorrhagic shock and was transferred to the SICU. Over the next days, her creatinine rose from 1.3 to 6.1 requiring dialysis and persistently required both platelet and RBC transfusions. She was transferred to the MICU where PLEX and high dose steroids were started. She was intubated for persistent altered mental status; video EEG showed status epilepticus and brain MRI showed multiple infarcts with hemorrhagic conversion. Her ADAMTS13 was <1%. She started Caplizumab and Rituximab which stabilized platelets. Despite treatment, her ADAMTS13 remained <1% by POD21 and she was started on Bortezumab. Blood Smear This is a severe case of TTP. There was early hesitation on the diagnosis - initially HELLP, then TTP vs. aHUS - given the degree of renal injury. PLEX was started along with high dose steroids, after which ADAMTS13 resulted. Upon the diagnosis of TTP, the platelet goal of 50,000 (for bleeding) was counterintuitively revised to 10,000 - as further platelets would serve as a nidus for clots and cause further strokes. Given her persistently low platelets, the decision was made to treat her aggressively with twice daily PLEX and starting Caplizumab to prevent any further clotting as well as Rituximab to reduce the immune system (which both had to be timed after PLEX to prevent removal of drug with PLEX) - demonstrating advanced and further therapy that can be used to treat refractory TTP. While the patient overall continued to improve, her ADAMTS13 continued to be < 1%, and she was started on Bortezumab. As of the time of writing, the patient is still in the hospital recovering. This abstract is funded by: None