{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T02:20:21Z","timestamp":1772504421281,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,11,7]],"date-time":"2024-11-07T00:00:00Z","timestamp":1730937600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Clinical Apheresis"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>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\u2010year\u2010old 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\u2010occurring 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.<\/jats:p>","DOI":"10.1002\/jca.22154","type":"journal-article","created":{"date-parts":[[2024,11,8]],"date-time":"2024-11-08T05:25:25Z","timestamp":1731043525000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Therapeutic Plasma Exchange Management for a Pediatric Patient Presenting With Immune Thrombotic Thrombocytopenic Purpura in a Setting of Common Variable Immunodeficiency"],"prefix":"10.1002","volume":"39","author":[{"given":"Albert","family":"Budhipramono","sequence":"first","affiliation":[{"name":"Department of Pathology University of Texas Southwestern Medical Center  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruchika","family":"Sharma","sequence":"additional","affiliation":[{"name":"Children's Health System  Dallas Texas USA"},{"name":"Department of Pediatrics, Division of Hematology and Oncology University of Texas Southwestern Medical Center  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian Allen","family":"Wysocki","sequence":"additional","affiliation":[{"name":"Children's Health System  Dallas Texas USA"},{"name":"Department of Pediatrics, Division of Allergy and Immunology University of Texas Southwestern Medical Center  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayesha N.","family":"Zia","sequence":"additional","affiliation":[{"name":"Children's Health System  Dallas Texas USA"},{"name":"Department of Pediatrics, Division of Hematology and Oncology University of Texas Southwestern Medical Center  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-7961-0083","authenticated-orcid":false,"given":"Brian D.","family":"Adkins","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Texas Southwestern Medical Center  Dallas Texas USA"},{"name":"Children's Health System  Dallas Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,11,7]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM195901012600104"},{"key":"e_1_2_7_3_1","unstructured":"\u201cClinical Working Party Diagnostic criteria for PID \u201d European Society for Immunodeficienciesaccessed March 4 2024 https:\/\/esid.org\/Working\u2010Parties\/Clinical\u2010Working\u2010Party\/Resources\/Diagnostic\u2010criteria\u2010for\u2010PID2."},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4725"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/35097008"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2352-3026(16)30018-7"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.13571"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-377945"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199108083250604"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-10-559211"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-196603000-00003"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25398"},{"key":"e_1_2_7_13_1","unstructured":"M. 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