Abstract Number: PO161
Meeting: ISTH 2021 Congress
Theme: Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies » ADAMTS13 and TTP
Background: Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, Neurologic abnormalities, and acute renal insufficiency.
Aims: To present the diagnosis and management of a case of refractory thrombotic thrombocytopenic purpura.
Methods: Hematological and biochemical studies were done .ADAMS 13 activity & antibodies were measured in our case to detect TTP.
Results: 27 year old lady, G3A2, at 21Weeks 6 days, was diagnosed to have Intrauterine fetal demise, so termination of pregnancy was done and she was advised LMWH for 6 weeks post-delivery. On PND 3 she presented with C/O giddiness and burning micturition.On evaluation her CBC was 6gm/dl and platelets were 20000/mm3. Peripheral smear showed schistocytes with thrombocytopenia. PT & APTT were normal. Empirical diagnosis of TTP was made and she was started on steroids and plasmapheresis. ADAMS 13 activity was <3 and ADAMTS inhibitor was >32. She received in total, 4 doses of Rituximab (weekly) & 2 doses of weekly iv vincristine (2mg) along with 29 cycles of plasmapheresis and was discharged when her platelet count was 1,66,000/mm3. She was again readmitted after 10 days with platelet count 18,000/mm3. Rpt ADAMTS activity was -<3 (n-68-163), ADAMTS inhibitor was >1.5 (n-<0.4). She received 5 cycles of plasmapheresis along with cyclosporine, which was added as a last resort. She was discharged on oral steroids and oral cyclosporin 100mg bd .Platelet count at the time of discharge was 2,05,000/mm3. She continues to be in remission post cyclosporine and is in follow up till now.
Conclusions: We present an unusual case of Thrombotic thrombocytopenic purpura, which was refactory to the usual treatments, i.e plasmapheresis ,rituximab and vincristine. In our case cyclosporine was effective along with plasmapheresis, so cyclosporine can be used in case of refractory TTP for remission.
To cite this abstract in AMA style:
Aribandi A, Ranjith C, Sushma S, Surapaneni T. Refractory Thrombotic Thrombocytopenic Purpura – A Case Report [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/refractory-thrombotic-thrombocytopenic-purpura-a-case-report/. Accessed May 20, 2022.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/refractory-thrombotic-thrombocytopenic-purpura-a-case-report/