Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchange
Catastrophic antiphospholipid syndrome (CAPS) involves sudden multiorgan dysfunction from thrombosis due to antibodies that cause platelet activation and endothelial dysfunction. Treatment variably combines anticoagulation, corticosteroid use, therapeutic plasma exchange (TPE), and high-dose intrave...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2021-08-01
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| Series: | Platelets |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/09537104.2020.1802414 |
| _version_ | 1797684122327449600 |
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| author | Tyler E. James Leslie J. Martin Theodore E. Warkentin Mark A. Crowther |
| author_facet | Tyler E. James Leslie J. Martin Theodore E. Warkentin Mark A. Crowther |
| author_sort | Tyler E. James |
| collection | DOAJ |
| description | Catastrophic antiphospholipid syndrome (CAPS) involves sudden multiorgan dysfunction from thrombosis due to antibodies that cause platelet activation and endothelial dysfunction. Treatment variably combines anticoagulation, corticosteroid use, therapeutic plasma exchange (TPE), and high-dose intravenous immunoglobulin (IVIG). A 42-year-old male with antiphospholipid syndrome (APS) presented with severe thrombocytopenia, encephalopathy, cardiac ischemia, and acral purpuric cutaneous lesions. CAPS was identified and he received heparin infusion, methylprednisolone, and IVIG. On day 7 he developed new purpuric lesions on his right foot despite detectable arterial pulses representing new microthrombosis refractory to IVIG. He was treated with TPE which resolved the right foot ischemia and eventually his CAPS. To our knowledge, this is the first patient with CAPS reported that failed initial treatment with IVIG and subsequently had excellent response to TPE. Our observations also support recent literature indicating that onset of thrombocytopenia in APS is a warning of progression to CAPS requiring treatment escalation. |
| first_indexed | 2024-03-12T00:26:03Z |
| format | Article |
| id | doaj.art-362caefd937a4417ba98c987b7142d5b |
| institution | Directory Open Access Journal |
| issn | 0953-7104 1369-1635 |
| language | English |
| last_indexed | 2024-03-12T00:26:03Z |
| publishDate | 2021-08-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Platelets |
| spelling | doaj.art-362caefd937a4417ba98c987b7142d5b2023-09-15T10:38:08ZengTaylor & Francis GroupPlatelets0953-71041369-16352021-08-0132682883110.1080/09537104.2020.18024141802414Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchangeTyler E. James0Leslie J. Martin1Theodore E. Warkentin2Mark A. Crowther3McMaster UniversityMcMaster UniversityMcMaster UniversityMcMaster UniversityCatastrophic antiphospholipid syndrome (CAPS) involves sudden multiorgan dysfunction from thrombosis due to antibodies that cause platelet activation and endothelial dysfunction. Treatment variably combines anticoagulation, corticosteroid use, therapeutic plasma exchange (TPE), and high-dose intravenous immunoglobulin (IVIG). A 42-year-old male with antiphospholipid syndrome (APS) presented with severe thrombocytopenia, encephalopathy, cardiac ischemia, and acral purpuric cutaneous lesions. CAPS was identified and he received heparin infusion, methylprednisolone, and IVIG. On day 7 he developed new purpuric lesions on his right foot despite detectable arterial pulses representing new microthrombosis refractory to IVIG. He was treated with TPE which resolved the right foot ischemia and eventually his CAPS. To our knowledge, this is the first patient with CAPS reported that failed initial treatment with IVIG and subsequently had excellent response to TPE. Our observations also support recent literature indicating that onset of thrombocytopenia in APS is a warning of progression to CAPS requiring treatment escalation.http://dx.doi.org/10.1080/09537104.2020.1802414catastrophic antiphospholipid syndromehigh-dose intravenous immunoglobulinmicrovascularthrombosistherapeutic plasma exchangethrombocytopeniathrombosis |
| spellingShingle | Tyler E. James Leslie J. Martin Theodore E. Warkentin Mark A. Crowther Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchange Platelets catastrophic antiphospholipid syndrome high-dose intravenous immunoglobulin microvascular thrombosis therapeutic plasma exchange thrombocytopenia thrombosis |
| title | Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchange |
| title_full | Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchange |
| title_fullStr | Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchange |
| title_full_unstemmed | Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchange |
| title_short | Catastrophic antiphospholipid syndrome refractory to high-dose intravenous immunoglobulin responsive to therapeutic plasma exchange |
| title_sort | catastrophic antiphospholipid syndrome refractory to high dose intravenous immunoglobulin responsive to therapeutic plasma exchange |
| topic | catastrophic antiphospholipid syndrome high-dose intravenous immunoglobulin microvascular thrombosis therapeutic plasma exchange thrombocytopenia thrombosis |
| url | http://dx.doi.org/10.1080/09537104.2020.1802414 |
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