Systemic Lupus Erythematosus with Concurrent Thrombotic Thrombocytopenic Purpura: A Case Report of Diagnostic Challenges and Management Approaches
Thrombotic Thrombocytopenic Purpura (TTP) is a thrombotic microangiopathy in which decreased activity of the von Willebrand Factor (vWF) multimer-cleaving protease ADAMTS13 leads to microvascular thrombosis. This condition can manifest as a pentad of symptoms that includes fever, thrombocytopenia, M...
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| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Private Limited
2025-02-01
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| Series: | Journal of Clinical and Diagnostic Research |
| Subjects: | |
| Online Access: | https://jcdr.net/articles/PDF/20583/76863_CE[Ra1]_F(SL)_QC(PS_SS)_PF1(AG_SS)_PFA(IS)_PB(AG_IS)_PN(IS).pdf |
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| Summary: | Thrombotic Thrombocytopenic Purpura (TTP) is a thrombotic microangiopathy in which decreased activity of the von Willebrand Factor (vWF) multimer-cleaving protease ADAMTS13 leads to microvascular thrombosis. This condition can manifest as a pentad of symptoms that includes fever, thrombocytopenia, Microangiopathic Haemolytic Anemia (MAHA), renal disease and neurological disease. Authors hereby, detail the case of a 29-year-old female with a rare combination of TTP and Systemic Lupus Erythematosus (SLE), which presents challenges in diagnosis, clinical progression and therapeutic response. The patient had an unusual presentation of a ruptured ovarian cyst with haemoperitoneum, which was surgically treated with emergency laparotomy. This patient also presented with a malar rash, confusion, thrombocytopenia and haematuria, raising suspicion for SLE, which was confirmed with positive autoantibodies. Continued neurological deterioration led to subdural haemorrhage with generalised tonic-clonic seizures and refractory severe thrombocytopenia, treatment with intravenous methylprednisolone, suggesting an underlying haematological condition. Clinical findings, including schistocytes on peripheral smear, elevated lactate dehydrogenase and indirect bilirubin, prompted investigation for ADAMTS13 activity. |
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| ISSN: | 2249-782X 0973-709X |