[Caplacizumab combined with therapeutic plasma exchange, glucocorticoids, and rituximab for refractory thrombotic thrombocytopenic purpura: a case report and literature review].

The clinical course and management of a patient with refractory immune thrombotic thrombocytopenic purpura (iTTP) admitted to the Department of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, were retrospectively analyzed, alongside a review of pertinent literature. Upon admission, the patient had a PLASMIC score of 6, and emergency therapeutic plasma exchange (TPE) combined with glucocorticoid therapy was immediately initiated. Further examinations revealed an ADAMTS13 activity of 2.9% and a positive inhibitor, thereby confirming iTTP diagnosis. After 5 consecutive days of TPE and glucocorticoid treatment, the patient demonstrated no clinical response, consistent with a diagnosis of refractory iTTP. Rituximab was subsequently added; however, the platelet count continued to decrease, and the disease progressed. After adding caplacizumab to the original regimen, the platelet and lactate dehydrogenase levels rapidly normalized, ADAMTS13 activity recovered to 24.9%, and no serious adverse events occurred during the treatment. This case indicates that incorporating caplacizumab into the combination therapy strategy for patients with refractory iTTP who failed conventional therapies effectively achieves rapid disease control.