Acquired idiopathic thrombotic thrombocytopenic purpura successfully treated with intravenous immunoglobulin and glucocorticoid

Abstract

Rationale:Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare.Patient concerns:A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia.Diagnosis:Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level.Interventions & Outcomes:Fresh frozen plasma was initially effective. Prednisolone and immunoglobulin resolved the condition with no adverse effects and rendered plasma exchange unnecessary.Lessons:Compared with biological agents, immunoglobulin is cost-effective, readily available, and has a proven long-term safety record, making it a possible treatment option for acquired thrombotic thrombocytopenic purpura.

Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review

“…PEX has not been systematically investigated in ASyS, but clinical improvement has been reported in combination with immunosuppressive drugs in some cases of severe ILD [ 18 ]. IVIG therapy, in our case introduced for myositis, has also been reported to be effective iTTP [ 19 ] as demonstrated by our case. Specifically, an inhibitory effect on ADAMTS13 antibodies by immunoglobulins is discussed.…”

Complete recovery of deep venous thrombosis from Coombs (+) thrombotic thrombocytopenic purpura: case report

“…Although the PEX-based method is the recommended acquired TTP treatment worldwide [ 12 ], other options could be considered for treating recurring or refractory TTP cases, or when severe adverse effects related to PEX such as bleeding or thrombosis appear [ 13 ]. Since 2002, therapeutic interventions aiming to B-cell depletion and reduction of autoantibodies, with rituximab, appear very effective both as induction therapy for the initiation of remission, as well as maintenance therapy, some even advocate the use of rituximab as routine initial treatment together with PEX and corticosteroids [ 14 ]; however, the frequencies of severe neurologic abnormalities, exacerbations, and death have not changed, while the frequency of relapse has decreased [ 15 ].…”

Complete recovery of deep venous thrombosis from Coombs (+) Thrombotic Thrombocytopenic Purpura: Case report

“…Although the PEX-based method is the recommended acquired TTP treatment worldwide 12 , other options could be considered for treating recurring or refractory TTP cases, or when severe adverse effects related to PEX such as bleeding or thrombosis appear 13 . Since 2002, therapeutic interventions aiming to B-cell depletion and reduction of autoantibodies, with rituximab, appear very effective both as induction therapy for the initiation of remission, as well as maintenance therapy, some even advocate the use of rituximab as routine initial treatment together with PEX and corticosteroids 14 ; however, the frequencies of severe neurologic abnormalities, exacerbations, and death have not changed, while the frequency of relapse has decreased 15 .…”