Abstract
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a life-threatening microvascular thrombotic disorder characterized by microangiopathic haemolytic anaemia, thrombocytopenia, and small vessel ischaemia. Current management of acute iTTP involves plasma exchange (PEX), (methyl)prednisolone, and caplacizumab. Although mortality rates decreased from >90% to ~10%, relapses occur in 30%–50% of the patients. Treatment of relapse or refractory iTTP is challenging and mostly consists of additional immunosuppressive therapy (such as rituximab, ciclosporin, vincristine, cyclophosphamide, and other T-cell modulating agents), or splenectomy.
iTTP is caused by a severe deficiency of ADAMTS13 activity due to IgG autoantibodies produced by B cells. Plasma-blasts and -cells do not express CD20, thereby escaping anti-CD20 therapy. However, they strongly express CD38. Several cases have been reported recently regarding the successful use of daratumumab, a humanized anti-CD38 antibody in refractory iTTP patients.10–12 Here, we present a refractory iTTP patient after multiple previous treatments including a recent splenectomy who was subsequently treated with daratumumab. Based on a systematic literature review, we have summarized characteristics of all previously described iTTP patients treated with daratumumab. [...]
iTTP is caused by a severe deficiency of ADAMTS13 activity due to IgG autoantibodies produced by B cells. Plasma-blasts and -cells do not express CD20, thereby escaping anti-CD20 therapy. However, they strongly express CD38. Several cases have been reported recently regarding the successful use of daratumumab, a humanized anti-CD38 antibody in refractory iTTP patients.10–12 Here, we present a refractory iTTP patient after multiple previous treatments including a recent splenectomy who was subsequently treated with daratumumab. Based on a systematic literature review, we have summarized characteristics of all previously described iTTP patients treated with daratumumab. [...]
Original language | English |
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Pages (from-to) | 2515-2518 |
Number of pages | 4 |
Journal | British Journal of Haematology |
Volume | 205 |
Issue number | 6 |
DOIs |
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Publication status | Published - 20 Oct 2024 |