Thrombotic thrombocytopenic purpura (TTP) is an uncommon life-threatening condition characterized by hemolytic disorders. The coexistence of systemic lupus erythematosus (SLE) with TTP is extremely rare, although Africans are at increased risk due to inherited risk factors. This report describes a rare clinical manifestation of TTP associated with SLE in a Sudanese patient. A 41-year-old Sudanese woman presented to the emergency department with symptoms and features that were suggestive of malaria, for which she had been treated accordingly. However, a few days later she complained of fever, and was found to have a body temperature of 39.5°C, jaundice, anemia, and thrombocytopenia. Soon after admission, she developed confusion and unrecordable blood pressure. After the patient had stabilized, clinical assessment, immune-system investigation (antinuclear antibody profile, complements, blood panel), and imaging revealed a diagnosis of TTP associated with SLE. The patient received imipenem 500 mg, five sessions of plasmapheresis (60 mL/kg), methylprednisolone 1 g pulse for 3 days, and rituximab 375 mg/week. Three weeks later, the patient was discharged after her condition had improved, and she is now on regular follow-up.
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http://dx.doi.org/10.2147/JBM.S334689 | DOI Listing |
Blood Adv
January 2025
Sanquin, Amsterdam, Netherlands.
In Immune-mediated Thrombotic Thrombocytopenic Purpura (iTTP), patients develop antibodies against ADAMTS13. The majority of patients exhibit inhibitory anti-spacer antibodies. Non-inhibitory antibodies binding to the carboxy-terminal CUB domains have been suggested to enhance the clearance of ADAMTS13 in iTTP.
View Article and Find Full Text PDFJ Immunother Precis Oncol
February 2025
Section of Benign Hematology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Thrombotic thrombocytopenic purpura (TTP) is characterized by thrombotic microangiopathy resulting from decreased activation of the von Willebrand factor-cleaving protease (ADAMTS13). TTP can cause organ damage and is often fatal if the appropriate treatment is not started immediately. Although primary immune TTP is the most common form of TTP, secondary immune etiologies, including complications from immune checkpoint inhibitors (ICIs), have also been reported.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, Hospital Beatriz Ângelo, Lisboa, PRT.
Vitamin B12 deficiency is a potentially severe condition with clinical manifestations ranging from nonspecific symptoms, such as asthenia and glossitis, to severe hematological problems, including pancytopenia and megaloblastic anemia. One of the rare phenomena associated with this condition is pseudo-thrombotic microangiopathy (pseudo-TMA), which can mimic diseases such as thrombotic thrombocytopenic purpura (TTP), leading to possible misdiagnosis and inappropriate treatment. In this article, we present the case of a 62-year-old man with a history of intravenous drug use, untreated hepatitis C, smoking, and alcoholism.
View Article and Find Full Text PDFSci Rep
January 2025
Discovery3 Team, Department of Research and Early Development, GC Biopharma, 93, Ihyeon-ro 30Beon-gil, Giheung-gu, Yongin-si, Gyeonggi-do, South Korea.
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening blood disorder characterized by the formation of blood clots in small blood vessels. It is caused by antibodies targeting the A disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13), which plays a role in cleaving von Willebrand factor. Most patients with iTTP have autoantibodies against specific domains of the ADAMTS13 protein, particularly the cysteine-rich and spacer domains.
View Article and Find Full Text PDFMedicine (Baltimore)
January 2025
Faculty of Chinese Medicine, Macau University of Science and Technology, Taipa, Macau, China.
Rationale: Thrombotic microangiopathies (TMA) caused by malignant hypertension is an acute and critical disease among rare diseases. Although renal biopsy pathology is a golden indicator for diagnosing kidney disease, it cannot distinguish between primary and secondary TMA and requires a comprehensive diagnosis in conjunction with other laboratory tests and medical history.
Patient Concerns: A 33-year-old young man was hospitalized due to unexplained kidney failure.
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