Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib
Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microt...
Saved in:
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2017-01-01
|
| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2017/9681832 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832556482581757952 |
|---|---|
| author | Manu R. Pandey Pankit Vachhani Evelena P. Ontiveros |
| author_facet | Manu R. Pandey Pankit Vachhani Evelena P. Ontiveros |
| author_sort | Manu R. Pandey |
| collection | DOAJ |
| description | Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microthrombi ultimately causing microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. Plasma exchange (PEX) with or without steroids constitutes standard first-line therapy with rituximab typically reserved for refractory cases. Therapies beyond rituximab lack strong evidence for routine use. Recently, bortezomib, a proteasome inhibitor used commonly in patients with multiple myeloma, was shown to induce remission in patients with refractory TTP. Here, we report a case of severe, relapsed TTP that was refractory to PEX, steroids, and rituximab that underwent remission following three cycles of bortezomib. We discuss the salient features of our case, the mechanism of action of bortezomib, and the very few other similar reports that exist in the literature. We conclude that bortezomib should be considered for patients with TTP refractory to PEX, steroids, and rituximab due to its efficacy and relatively favorable side effect profile. |
| format | Article |
| id | doaj-art-901d1d07a3e44cb7a666dbede176dabc |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-901d1d07a3e44cb7a666dbede176dabc2025-02-03T05:45:18ZengWileyCase Reports in Hematology2090-65602090-65792017-01-01201710.1155/2017/96818329681832Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of BortezomibManu R. Pandey0Pankit Vachhani1Evelena P. Ontiveros2Department of Internal Medicine, Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, USALeukemia Service, Department of Medicine, Roswell Park Cancer Institute, Buffalo, NY, USALeukemia Service, Department of Medicine, Roswell Park Cancer Institute, Buffalo, NY, USAAcquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microthrombi ultimately causing microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. Plasma exchange (PEX) with or without steroids constitutes standard first-line therapy with rituximab typically reserved for refractory cases. Therapies beyond rituximab lack strong evidence for routine use. Recently, bortezomib, a proteasome inhibitor used commonly in patients with multiple myeloma, was shown to induce remission in patients with refractory TTP. Here, we report a case of severe, relapsed TTP that was refractory to PEX, steroids, and rituximab that underwent remission following three cycles of bortezomib. We discuss the salient features of our case, the mechanism of action of bortezomib, and the very few other similar reports that exist in the literature. We conclude that bortezomib should be considered for patients with TTP refractory to PEX, steroids, and rituximab due to its efficacy and relatively favorable side effect profile.http://dx.doi.org/10.1155/2017/9681832 |
| spellingShingle | Manu R. Pandey Pankit Vachhani Evelena P. Ontiveros Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib Case Reports in Hematology |
| title | Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib |
| title_full | Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib |
| title_fullStr | Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib |
| title_full_unstemmed | Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib |
| title_short | Remission of Severe, Relapsed, and Refractory TTP after Multiple Cycles of Bortezomib |
| title_sort | remission of severe relapsed and refractory ttp after multiple cycles of bortezomib |
| url | http://dx.doi.org/10.1155/2017/9681832 |
| work_keys_str_mv | AT manurpandey remissionofsevererelapsedandrefractoryttpaftermultiplecyclesofbortezomib AT pankitvachhani remissionofsevererelapsedandrefractoryttpaftermultiplecyclesofbortezomib AT evelenapontiveros remissionofsevererelapsedandrefractoryttpaftermultiplecyclesofbortezomib |