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Thrombotic thrombocytopenic purpura
Charlotte Kaiser1, Ulrich Gembruch, Viktor Janzen
1Department of Obstetrics and Prenatal Medicine, University Clinics, Bonn, Germany.
Summary
This case study highlights a rare instance of thrombotic thrombocytopenic purpura (TTP) in a pregnant patient, complicated by HELLP syndrome. Prompt plasma exchange successfully treated TTP, leading to recovery.
Area of Science:
- Obstetrics and Gynecology
- Hematology
- Critical Care Medicine
Background:
- A 34-year-old primipara presented with dichorionic-diamniotic twins at 26+3 weeks gestation.
- Initial suspicion of HELLP syndrome led to a cesarean section at 27+4 weeks due to severe thrombocytopenia (platelets 44,000/µl).
Observation:
- Post-operative re-laparotomy was required for intra-abdominal bleeding, with the patient receiving platelet and erythrocyte transfusions.
- The patient developed blurred vision and facial nerve paresis, indicative of neurological compromise.
- Laboratory findings revealed persistent thrombocytopenia, elevated D-dimer, LDH, reticulocytes, and reduced haptoglobin, alongside reduced ADAMTS-13 activity.
Findings:
- Thrombotic thrombocytopenic purpura (TTP) was confirmed by reduced ADAMTS-13 activity.
- Plasma exchange was identified as the optimal treatment, with platelet concentrate transfusion advised against due to potential exacerbation of microangiopathy.
- Following plasma exchange, platelet counts normalized, and ophthalmic examination revealed choroid perfusion dysfunction as the cause of visual disturbances.
Implications:
- This case underscores the importance of considering TTP in pregnant patients presenting with severe thrombocytopenia and microangiopathic hemolytic anemia symptoms.
- Early diagnosis and appropriate management, particularly plasma exchange, are crucial for favorable maternal and neonatal outcomes in TTP during pregnancy.
- The successful management highlights the efficacy of plasma exchange in TTP, even in the complex obstetric setting, while emphasizing the risks associated with platelet transfusions.
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