Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura (TTP) is a rare form of thrombotic microangiopathy (TMA) typically characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and end-organ ischemia secondary to microvascular dissemination of platelet-rich thrombi. Systemic Lupus Erythematosus (...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-08-01
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| Series: | Thrombosis Update |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666572722000220 |
| _version_ | 1811340047879766016 |
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| author | Dariusz Uczkowski Hristos Milonas |
| author_facet | Dariusz Uczkowski Hristos Milonas |
| author_sort | Dariusz Uczkowski |
| collection | DOAJ |
| description | Thrombotic Thrombocytopenic Purpura (TTP) is a rare form of thrombotic microangiopathy (TMA) typically characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and end-organ ischemia secondary to microvascular dissemination of platelet-rich thrombi. Systemic Lupus Erythematosus (SLE) is a chronic autoimmune condition characterized by multi-organ system inflammation with the generation of autoantibodies. The specific cause of SLE is unknown but multiple factors seem to be associated with the development of the disease including: genetic, epigenetic, ethnic, hormonal, and environmental factors. In SLE, patients may present with a wide assortment of clinical variations, but diagnosis is based generally on clinical and laboratory findings after excluding alternative diagnoses. Very rarely do SLE and TTP present simultaneously. Both disease processes have overlapping clinical features which makes diagnosis and management challenging. The following case report describes a forty-year-old female with no prior history of SLE or TTP who presented with clinical findings and features of TTP as the initial clinical manifestation of her underlying SLE, as well as serologic criteria, requiring treatment with plasma exchange, high dose steroids, and eventual outpatient infusion with Rituximab. |
| first_indexed | 2024-04-13T18:36:35Z |
| format | Article |
| id | doaj.art-db4c5247f1954c93879ea4c17fea4570 |
| institution | Directory Open Access Journal |
| issn | 2666-5727 |
| language | English |
| last_indexed | 2024-04-13T18:36:35Z |
| publishDate | 2022-08-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Thrombosis Update |
| spelling | doaj.art-db4c5247f1954c93879ea4c17fea45702022-12-22T02:34:53ZengElsevierThrombosis Update2666-57272022-08-018100118Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic PurpuraDariusz Uczkowski0Hristos Milonas1Overlook Medical Center, Atlantic Health System, USAOverlook Medical Center, Atlantic Health System, USAThrombotic Thrombocytopenic Purpura (TTP) is a rare form of thrombotic microangiopathy (TMA) typically characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and end-organ ischemia secondary to microvascular dissemination of platelet-rich thrombi. Systemic Lupus Erythematosus (SLE) is a chronic autoimmune condition characterized by multi-organ system inflammation with the generation of autoantibodies. The specific cause of SLE is unknown but multiple factors seem to be associated with the development of the disease including: genetic, epigenetic, ethnic, hormonal, and environmental factors. In SLE, patients may present with a wide assortment of clinical variations, but diagnosis is based generally on clinical and laboratory findings after excluding alternative diagnoses. Very rarely do SLE and TTP present simultaneously. Both disease processes have overlapping clinical features which makes diagnosis and management challenging. The following case report describes a forty-year-old female with no prior history of SLE or TTP who presented with clinical findings and features of TTP as the initial clinical manifestation of her underlying SLE, as well as serologic criteria, requiring treatment with plasma exchange, high dose steroids, and eventual outpatient infusion with Rituximab.http://www.sciencedirect.com/science/article/pii/S2666572722000220SLETTPSchistocytesMicroangiopathic hemolytic anemiaPlasma exchange |
| spellingShingle | Dariusz Uczkowski Hristos Milonas Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura Thrombosis Update SLE TTP Schistocytes Microangiopathic hemolytic anemia Plasma exchange |
| title | Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura |
| title_full | Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura |
| title_fullStr | Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura |
| title_full_unstemmed | Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura |
| title_short | Initial presentation of Systemic Lupus Erythematosus coinciding with Thrombotic Thrombocytopenic Purpura |
| title_sort | initial presentation of systemic lupus erythematosus coinciding with thrombotic thrombocytopenic purpura |
| topic | SLE TTP Schistocytes Microangiopathic hemolytic anemia Plasma exchange |
| url | http://www.sciencedirect.com/science/article/pii/S2666572722000220 |
| work_keys_str_mv | AT dariuszuczkowski initialpresentationofsystemiclupuserythematosuscoincidingwiththromboticthrombocytopenicpurpura AT hristosmilonas initialpresentationofsystemiclupuserythematosuscoincidingwiththromboticthrombocytopenicpurpura |