Abstract Number: VPB0317
Meeting: ISTH 2022 Congress
Background: Thrombotic thrombocytopenic purpura (TTP) has high lethal rate what justified its early diagnosis and beginning of treatment.
Aims: To present the diagnosis and management of cases of patients with TTP.
Methods: We report 6 cases of patients from 23 to 46 years old with TTP. Hematological and biochemical tests were performed; activity of ADAMTS 13 was measured.
Results: All patients had sudden onset of the disease that manifested with neurologic symptoms: headache, speech impairment, numbness of a limb accompanied by progressive thrombocytopenia with hemorrhage and microangiopathic hemolytic anemia (MAHA). Nobody had all sings from TTP pentad. On admission all patients had hemoglobin (HGB) < 90 g/dL, thrombocytes < 26x109/L, reticulocytosis > 15%, mean leukocytes count – 10±2.2 x109/L. Schistocytes were described in 3 persons. Markers of MAHA were revealed with elevation of lactate dehydrogenase – 5703±2400 IU/mL, decline of haptoglobine – 2.1±0.5 mg/dL. ADAMTS 13 activity was measured in 3 patients and appeared to be 0%. The diagnosis of TTP set up in them and suspected in others patients. Pulse-therapy with steroids for 5 days and plasmapheresis – 18 procedures for each patient – were started immediately in 4 patients and stopped when thrombocytes were ≥ 120×109/L and decrease of MAHA intensity was reached. Stable remission was achieved in them. Other 2 patients had 4-day delay in the beginning of plasmapheresis. Their HGB level and thrombocytes started to rise only after 25th cycle of plasmapheresis. Rituximab once a week for 4 weeks was added. Unfortunately one patient developed hemorrhagic stroke and the other – secondary hemolytic-uremic syndrome and polyorgan failure. Four patients are followed up for a year now, no signs of relapse are found.
Conclusion(s): Setting up the diagnosis of TTP early is crucial for its treatment with plasmapheresis playing the mail role. It delay can significantly decrease survival rate.
To cite this abstract in AMA style:Talako T, Tsvirko D, Kabaeva K, Iskrov I, Statsenko Y, Avadok A, Lendina I. Experience in Diagnosis and Treatment of Thrombotic Thrombocytopenic Purpura – Case Reports [abstract]. https://abstracts.isth.org/abstract/experience-in-diagnosis-and-treatment-of-thrombotic-thrombocytopenic-purpura-case-reports/. Accessed September 29, 2023.
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