
Systemic Lupus Erythematosus with Concurrent Thrombotic Thrombocytopenic Purpura: A Case Report of Diagnostic Challenges and Management Approaches
Correspondence Address :
Mr. Pruthvi Raj Kukunoor,
H. No: 14-13, Shivajinagar, Kapra, Jawaharnagar-500087, Telangana, India.
E-mail: kukunoorpruthviraj@gmail.com
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.
Autoimmune disease, Haematologic disorder, Refractory disease
DOI: 10.7860/JCDR/2025/76863.20583
Date of Submission: Nov 20, 2024
Date of Peer Review: Dec 16, 2024
Date of Acceptance: Jan 07, 2025
Date of Publishing: Feb 01, 2025
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Nov 23, 2024
• Manual Googling: Dec 20, 2024
• iThenticate Software: Jan 04, 2025 (3%)
ETYMOLOGY: Author Origin
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