Combined therapy aids recovery for woman with TTP and lupus
Case shows early recognition of TTP key to preventing complications
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Recognizing thrombotic thrombocytopenic purpura (TTP) in a woman with lupus and starting combined treatment — Cablivi (caplacizumab) on top of plasma exchange, corticosteroids, and rituximab — led to her rapid recovery and may have prevented serious complications, according to a report from the University of Arizona College of Medicine.
The report, “Caplacizumab in Lupus-Associated Thrombotic Thrombocytopenic Purpura (TTP): Navigating Autoimmunity and Microangiopathy,” was published in the Cureus Journal of Medical Sciences.
Platelets in small vessels form blood clots that restrict blood supply to the body’s organs, leading to TTP symptoms. In immune-mediated TTP, also known as acquired TTP, this occurs because antibodies mistakenly attack ADAMTS13, an enzyme that prevents the formation of unnecessary blood clots.
While anyone can develop TTP, certain risk factors may make it more likely to occur. The researchers described the case of a 55-year-old woman with Sjögren’s disease and a recent diagnosis of systemic lupus erythematosus (SLE), another autoimmune disease, who was later also diagnosed with TTP.
“This report highlights the need to recognize immune TTP as a potential complication in patients with SLE,” the researchers wrote. “Additionally, it emphasizes the importance of timely intervention and early use of available treatments, particularly [Cablivi], to optimize clinical outcomes.”
Symptoms prompt hospital visit
The woman arrived at the hospital after a month of worsening bruising, weakness, dizziness, shortness of breath, and chest and back pain. She had dry eyes and mouth from Sjögren’s and had not been taking her prescribed hydroxychloroquine (a common medication for inflammatory conditions).
Initial blood tests showed severe anemia (low levels of red blood cells or of the hemoglobin protein that carries oxygen) and very low platelets. A blood smear revealed numerous schistocytes, which are broken fragments of red blood cells, and increased markers of hemolysis, the destruction of red blood cells.
Because she had both low platelets and signs of hemolysis, doctors measured ADAMTS13 activity. It was markedly low, “confirming the diagnosis of TTP.” Repeat testing detected an ADAMTS13 inhibitor, meaning the immune system was actively blocking the enzyme through a neutralizing antibody.
Imaging studies showed enlarged lymph nodes but no major damage to the woman’s organs. Infections were ruled out. Using diagnostic criteria for lupus, the woman met the classification for SLE and had risk scores indicating intermediate to high likelihood of TTP.
Doctors immediately began treatment with plasma exchange. This procedure removes a patient’s plasma, which contains disease-causing antibodies, and replaces it with fresh donor plasma to restore ADAMTS13. Treatment also included high-dose corticosteroids to suppress the immune system and reduce inflammation.
Clinicians added rituximab, marketed as Rituxan and biosimilars, because the woman had active lupus and an ADAMTS13 inhibitor. Rituximab reduces the number of B-cells, the immune cells that produce antibodies. Hydroxychloroquine was restarted as part of a long-term strategy to control lupus.
The woman’s platelet count initially increased, but dropped again over the following weeks. At that point, she was started on Cablivi. This medication, from Sanofi, binds to a protein called von Willebrand factor to help prevent the formation of blood clots.
Her symptoms eased after starting Cablivi. Her anemia resolved, and her platelets were near normal. Markers of hemolysis also normalized. This confirmed the effectiveness of combined treatment with plasma exchange, corticosteroids, rituximab, and Cablivi.
The case illustrates how early recognition and prompt treatment are crucial in TTP, the researchers said. “Early ADAMTS13 testing and timely incorporation of [Cablivi] may help reduce mortality in autoimmune TTP, including lupus-associated cases,” they wrote.
