Refractory Acquired Thrombotic Thrombocytopenic Purpura in the Third Trimester of Twin Pregnancy: A Case Report

Jeya Sushimitha Jawahar Kanth,1 Morrish Obol Okello,2,3 Thuparambil M Ravi Anish,1 Micheal Collins Segawa,4 Herbert Kayiga,1 Sheethal Madari51Department of Obstetrics and Gynecology, School of Medicine, College of Health Sciences, Makerere University, Kampala, Uganda; 2Faculty of Medicine, Gulu University, Gulu, Uganda; 3Department of Pediatrics and Child Health, Jinja Regional Referral Hospital, Jinja, Uganda; 4School of Medicine, College of Health Sciences, Makerere University, Kampala, Uganda; 5Department of Obstetrics and Gynecology, School of Medicine, College of Health Sciences, Aberdeen University, Aberdeen, UKCorrespondence: Morrish Obol Okello, Faculty of Medicine, Gulu University, P.O. Box 166, Gulu, Uganda, Tel +256778633788, Email [email protected]: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening condition triggered by pregnancy, often in the third trimester, and frequently mimics preeclampsia or HELLP syndrome due to shared features like thrombocytopenia. Refractory cases in twin pregnancies, described in this case, pose unique diagnostic and therapeutic challenges, requiring advanced interventions.Case Presentation: A 29-year-old woman carrying dichorionic-diamniotic twins presented in the third trimester with palpitations, dyspnea, oral bleeding, numbness, chest heaviness, fatigue, edema, and pelvic pain. Admission showed severe thrombocytopenia (8 × 109/L), microangiopathic hemolytic anemia (schistocytes, elevated LDH), normal blood pressure, and normal liver/kidney function, making preeclampsia/HELLP unlikely. Because of the high bleeding risk posed by the profound thrombocytopenia, and despite a reassuring fetal assessment, an emergency cesarean section was performed and delivered two healthy baby girls with good Apgar scores. ADAMTS13 activity < 10% subsequently confirmed acquired TTP. Plasma exchange and corticosteroids were started presumptively on the day of admission; because the platelet response was incomplete, rituximab and caplacizumab were added during the same admission. The patient was discharged after initial hematologic recovery but relapsed with recurrent severe thrombocytopenia three days later, confirming refractory disease; she was readmitted and achieved sustained platelet normalization with continued plasma exchange, rituximab, and caplacizumab. Complications included wound infection with multiple organisms, antibiotic allergy, and superficial venous thrombosis, all managed successfully. Both mother and infants were discharged well, with no further relapse at six-week follow-up. Future pregnancies were discouraged without specialist review.Conclusions: High suspicion for TTP is essential in pregnant patients with thrombocytopenia and hemolysis, even without hypertension. Rapid ADAMTS13 testing, timely delivery, and combined plasma exchange with rituximab and caplacizumab enabled full recovery in this refractory twin-pregnancy case. Multidisciplinary care is critical to differentiate from obstetric mimics and achieve favorable outcomes.Keywords: TTP, twin pregnancy, ADAMTS13, caplacizumab, case report