Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against the A subunit of FXIII, which interfere with different functions of FXIII, have been described. Here, for the first time, we report an autoantibody against the B subunit of FXIII (FXIII-B) that caused life-threatening bleeding in a patient with systemic lupus erythematosus. FXIII activity, FXIII-A(2)B(2) complex, and individual FXIII subunits were undetectable in the plasma, whereas platelet FXIII activity and antigen were normal. Neither FXIII activation nor its activity was inhibited by the antibody, which bound to structural epitope(s) on both free and complexed FXIII-B. The autoantibody highly accelerated the elimination of FXIII from the circulation. FXIII supplementation combined with immunosuppressive therapy, plasmapheresis, immunoglobulin, and anti-CD20 treatment resulted in the patient's recovery. FXIII levels returned to around 20% at discharge and after gradual increase the levels stabilized above 50%.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1182/blood-2008-09-179333 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Background: Germline haplodeficiency (RHD) is associated with thrombocytopenia, platelet dysfunction and predisposition to myeloid malignancies. Platelet expression profiling of a RHD patient showed decreased encoding for the A subunit of factor XIII, a transglutaminase that cross-links fibrin and induces clot stabilization. FXIII-A is synthesized by hematopoietic cells, megakaryocytes and monocytes.
View Article and Find Full Text PDFEstablishing the reference intervals of plasma PLG, FXII activity, and FXIII antigen in healthy adults in Guangzhou.
BMC Cardiovasc Disord
January 2025
Department of Laboratory Medicine, Zhujiang Hospital, Southern Medical University, 253 Industrial Avenue Central, Guangzhou, 510280, P. R. China.
Objective: To establish the reference intervals of plasma Plasminogen, Factor XII activity, and Factor XIII Antigen in healthy adults in Guangzhou.
Methods: A total of 168 young people (75 males and 93 females, aged 18-65 years) who underwent physical examination in Zhujiang Hospital of Southern Medical University from 2020 to 2022 were recruited. Sysmex CS5100 automatic coagulation analyzer and matching reagents were used to detect Plasminogen.
Diagnosis, treatment, surgical practices and review of the literature in rare coagulation factor deficiencies.
Ital J Pediatr
January 2025
Pediatric Hematology and Oncology, SBU Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey.
Background: Rare bleeding disorders (RBDs) include fibrinogen (Factor I), prothrombin (Factor II), Factor V(FV), combined Factor V and Factor VIII, Factor VII, Factor X, Factor XI, Factor XII, and Factor XIII deficiencies. This group accounts for 3-5% of all factor deficiencies. Different symptoms may occur, ranging from mild or moderate bleeding to serious and life-threatening bleeding, which may not be related to the factor level.
View Article and Find Full Text PDFProfile of haemostasis and coagulation in patients with peritoneal carcinomatosis undergoing cytoreductive surgery with hyperthermal chemotherapy.
Eur J Surg Oncol
December 2024
Critical Care Division, University Hospital 'Virgen Del Rocío', Avda Manuel Siurot S/n, 41013, Seville, Spain. Electronic address:
Background: One-third of patients with peritoneal carcinomatosis undergoing cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) present alterations in conventional coagulation test results. However, perioperative coagulation has not been systematically investigated in these patients. This study aimed to investigate hemostatic changes in such patients.
View Article and Find Full Text PDFUnderstanding the development of tuberculous granulomas: insights into host protection and pathogenesis, a review in humans and animals.
Front Immunol
December 2024
Department of Academic Affairs, National Jewish Health, Denver, CO, United States.
Granulomas, organized aggregates of immune cells which form in response to (), are characteristic but not exclusive of tuberculosis (TB). Despite existing investigations on TB granulomas, the determinants that differentiate host-protective granulomas from granulomas that contribute to TB pathogenesis are often disputed. Thus, the goal of this narrative review is to help clarify the existing literature on such determinants.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!