Clinical features and outcomes of thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency at Maharat Nakhon Ratchasima Hospital
Keywords:
Thrombotic thrombocytopenic purpura, Severe ADAMTS13 deficiency, Plasma exchangeAbstract
Abstract
Introduction: Thrombotic thrombocytopenia purpura (TTP) is a rare but serious disease that is characterized by the microangiopathic hemolytic anemia (MAHA), thrombocytopenia, neurological abnormalities, renal impairment and fever, the so-called pentad. However identifying cases who completely fulfill the pentad is unusual and its various clinical presentations cause difficult diagnosis and high mortality rate.
Objective: The study aimed to review clinical data, laboratory data, therapeutic interventions and treatment outcomes of patients with a diagnosis of TTP at Maharat Nakhon Ratchasima Hospital (MNRH) between January 2007 and June 2017.
Result: In all, 22 TTP patients received a definite diagnosis with severe ADAMTS13 deficiency. Their mean age was 59.8 years. Twenty were classified as idiopathic (91.0%) while two were SLE-associated TTP (9.0%). All patients had MAHA and thrombocytopenia, 93% had neurological presentations, and 45.4% had bleeding disorder. Their mean laboratory data included hemoglobin concentration, platelet, and creatinine level of 7.1 g/dL, 11.4 x109 /L and 1.7 mg/dL, respectively. Only 36.3% of patients had the full pentad of TTP. Their treatments at the time of diagnosis consisted of plasma exchange in 11, plasma infusion in 10 and only immunosuppressants in 1 patient. The mean interval between symptom onset and plasma exchange was 6.6 days. The complete remission rate was 50%. Two patients relapsed only within the first year, one could achieve complete remission again by plasma exchange with immunosuppressants but the other patient passed away. The overall mortality rate was 50.0%.
Conclusion: Almost all patients with TTP in our series had neurological symptoms clinically mimicking ischemic stroke, so the diagnosis could be delayed and might be the cause of the high mortality rate.
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References
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