CO-71 | ACQUIRED FACTOR XIII DEFICIENCY IN A PATIENT POST-CAR-T THERAPY FOR NON-HODGKIN LYMPHOMA: A CASE REPORT

A. Strangio, M. Leotta, A. Ierardi, R.C. Santoro | Hemophilia, Hemostasis and Thrombosis Unit, Azienda Ospedaliero Universitaria Renato Dulbecco, Catanzaro, Italy

Introduction. Factor XIII-A (FXIII-A) deficiency is an ultra-rare bleeding disorder characterized by high rates of morbidity and mortality, primarily resulting from intracranial hemorrhage, umbilical cord bleeding, and miscarriage, whereas patients with severe FXIII-B deficiency present with a milder phenotype. individuals with heterozygous FXIII- A deficiency, particularly in women, is more likely to result in hemorrhagic complications during hemostatic challenges. Chimeric antigen receptor (CAR)‑T cell therapy is an innovative approach to immune cell therapy that works by modifying the T cells of a patient to express the CAR protein on their surface and thus induce their recognition and destruction of cancer cells. CAR-T therapy has emerged as a promising treatment for lymphoid malignancies, including lymphomas. During treatment, coagulation abnormalities, which are prevalent complications in CAR-T cell therapy, can range in severity from simple abnormalities in coagulation parameters to serious hemorrhage or disseminated intravascular coagulation associated with life-threatening multiorgan dysfunction. However, it is not known whether coagulation alterations may be present or persist several months after treatment, especially with regard to potential acquired deficiencies.
Case Presentation. We report a 29-year-old female patient with a history of lymphoma (LNH) successfully treated with CAR-T therapy, achieving remission. Notably, her medical history revealed no prior bleeding episodes. Recently, she presented with cutaneous hemorrhages and increased menstrual bleeding, raising suspicion for an acquired coagulation disorder. A complete blood count was performed, which returned normal results, along with a comprehensive coagulation screening test, which also yielded normal results. In addition, a comprehensive screening for von Willebrand was conducted and returned normal results. However, the FXIII assay indicated a reduced FXIII level (4%). Screening of both parents showed normal FXIII levels, further complicating the clinical picture. Following fears of a relapse of the disease, in-depth evaluations were carried out, which ruled out a recurrence of the lymphoma. Four weeks after the initial evaluation, laboratory tests confirmed reduced levels of FXIII, with persistent increased menstrual bleeding and mild cutaneous hemorrhages.
Discussion. The patient's condition highlights the importance of monitoring coagulation factors in patients undergoing CAR-T therapy, as acquired deficiencies can occur without prior bleeding history. The patient continues to be under close observation, and genetic testing is pending to explore potential underlying causes of her FXIII deficiency.