Therapeutic Plasma Exchange Management for a Pediatric Patient Presenting With Immune Thrombotic Thrombocytopenic Purpura in a Setting of Common Variable Immunodeficiency.

Autor: Budhipramono A; Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA., Sharma R; Children's Health System, Dallas, Texas, USA.; Department of Pediatrics, Division of Hematology and Oncology, University of Texas Southwestern Medical Center, Dallas, Texas, USA., Wysocki CA; Children's Health System, Dallas, Texas, USA.; Department of Pediatrics, Division of Allergy and Immunology, University of Texas Southwestern Medical Center, Dallas, Texas, USA., Zia AN; Children's Health System, Dallas, Texas, USA.; Department of Pediatrics, Division of Hematology and Oncology, University of Texas Southwestern Medical Center, Dallas, Texas, USA., Adkins BD; Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.; Children's Health System, Dallas, Texas, USA.
Jazyk: angličtina
Zdroj: Journal of clinical apheresis [J Clin Apher] 2024 Dec; Vol. 39 (6), pp. e22154.
DOI: 10.1002/jca.22154
Abstrakt: Common variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia resulting in recurrent infections. While autoimmune disorders are common in patients with CVID, no association has been reported between CVID and immune thrombotic thrombocytopenic purpura (iTTP), a disorder most often caused by autoantibodies that compromise the activity of the enzyme ADAMTS13. Reduced ADAMTS13 activity results in the accumulation of large von Willebrand factor multimers that can consume platelets and cause microvascular thrombosis and organ injury, ultimately resulting in mortality in most cases of untreated iTTP. Here, we report a 12-year-old male with CVID who developed iTTP, underwent therapeutic plasma exchange (TPE), and subsequently recovered. We conducted a systematic review for other cases of CVID co-occurring with iTTP and present additional cases of this rare presentation. We highlight the importance of prompt recognition of iTTP in a patient with CVID and timely initiation of TPE.
(© 2024 The Author(s). Journal of Clinical Apheresis published by Wiley Periodicals LLC.)
Databáze: MEDLINE
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