{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T22:48:06Z","timestamp":1775083686580,"version":"3.50.1"},"reference-count":21,"publisher":"The Endocrine Society","issue":"4","license":[{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"vor","delay-in-days":13,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,3,19]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The idiopathic multicentric Castleman disease (CD)\u2013TAFRO variant (iMCD-TAFRO) is a rare, clinically aggressive lymphoproliferative disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis\/renal failure, and organomegaly. Associated with elevated inflammatory cytokines, iMCD-TAFRO can present severe systemic complications. Here, we report a previously healthy adolescent male presenting with severe systemic inflammation, diffuse lymphadenopathy, anasarca, renal impairment, and hepatosplenomegaly. His clinical course was complicated by suspected bilateral adrenal infarction leading to primary adrenal insufficiency (PAI) requiring glucocorticoid (GC) replacement and new-onset autoimmune thyroiditis (AIT) requiring thyroid hormone replacement, suggesting a broader autoimmune process. Diagnosis of iMCD-TAFRO was supported by a right iliac lymph node biopsy showing CD features. The patient was successfully treated with high-dose GC followed by targeted immunosuppressive therapy with siltuximab, an anti-interleukin-6 monoclonal antibody. This case highlights a rare presentation of iMCD-TAFRO syndrome in a pediatric patient with unique co-occurrence of 2 endocrinopathies; PAI and AIT. Prompt recognition of potential associated endocrinopathies and a multidisciplinary approach are critical for management and improved outcomes in this life-threatening condition.<\/jats:p>","DOI":"10.1210\/jcemcr\/luag006","type":"journal-article","created":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T12:34:15Z","timestamp":1768048455000},"source":"Crossref","is-referenced-by-count":0,"title":["Primary adrenal insufficiency and autoimmune thyroiditis in an adolescent with Castleman-TAFRO syndrome"],"prefix":"10.1210","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7247-9506","authenticated-orcid":false,"given":"Manahil","family":"Mustafa","sequence":"first","affiliation":[{"name":"Division of Endocrinology, Department of Pediatrics, Indiana University\/Riley Children's Hospital , IN ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vasantha D","family":"Aaron","sequence":"additional","affiliation":[{"name":"Division of Radiology, Department of Pediatrics, Indiana University\/Riley Children's Hospital , IN ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathleen","family":"Overholt","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology, Indiana University\/Riley Children's Hospital , IN ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander","family":"Tuttle","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Department of Pediatrics, Indiana University\/Riley Children's Hospital , IN ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","published-online":{"date-parts":[[2026,4,1]]},"reference":[{"issue":"1","key":"2026040117232334200_luag006-B1","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.hoc.2017.09.009","article-title":"TAFRO syndrome","volume":"32","author":"Igawa","year":"2018","journal-title":"Hematol Oncol Clin North Am"},{"issue":"1","key":"2026040117232334200_luag006-B2","doi-asserted-by":"crossref","first-page":"63","DOI":"10.3960\/jslrt.53.63","article-title":"Thrombocytopenia with reticulin fibrosis accompanied by fever, anasarca and hepatosplenomegaly: a clinical report of five cases","volume":"53","author":"Takai","year":"2013","journal-title":"J Clin Exp Hematop"},{"issue":"37","key":"2026040117232334200_luag006-B3","doi-asserted-by":"crossref","first-page":"E1262","DOI":"10.1503\/cmaj.240947","article-title":"TAFRO subtype of idiopathic multicentric Castleman disease in a 22-year-old man","volume":"196","author":"Rowe","year":"2024","journal-title":"CMAJ"},{"issue":"4","key":"2026040117232334200_luag006-B4","doi-asserted-by":"crossref","first-page":"837","DOI":"10.3390\/biomedicines12040837","article-title":"Radiological imaging findings of adrenal abnormalities in TAFRO syndrome: a systematic review","volume":"12","author":"Kurokawa","year":"2024","journal-title":"Biomedicines"},{"issue":"10","key":"2026040117232334200_luag006-B5","doi-asserted-by":"crossref","first-page":"5588","DOI":"10.1007\/s00330-020-06919-1","article-title":"Computed tomography findings of early-stage TAFRO syndrome and associated adrenal abnormalities","volume":"30","author":"Kurokawa","year":"2020","journal-title":"Eur Radiol"},{"issue":"18","key":"2026040117232334200_luag006-B6","doi-asserted-by":"crossref","first-page":"2615","DOI":"10.2169\/internalmedicine.2717-19","article-title":"Subclinical hypothyroidism in TAFRO syndrome","volume":"58","author":"Oka","year":"2019","journal-title":"Intern Med"},{"issue":"12","key":"2026040117232334200_luag006-B7","doi-asserted-by":"crossref","first-page":"1646","DOI":"10.1182\/blood-2016-10-746933","article-title":"International, evidence-based consensus diagnostic criteria for HHV-8-negative\/idiopathic multicentric Castleman disease","volume":"129","author":"Fajgenbaum","year":"2017","journal-title":"Blood"},{"issue":"6","key":"2026040117232334200_luag006-B8","doi-asserted-by":"crossref","first-page":"1328","DOI":"10.3390\/biomedicines12061328","article-title":"Increase in vascular endothelial growth factor (VEGF) expression and the pathogenesis of iMCD-TAFRO","volume":"12","author":"Srkalovic","year":"2024","journal-title":"Biomedicines"},{"issue":"1","key":"2026040117232334200_luag006-B9","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1186\/s13256-018-1814-9","article-title":"The first case of thrombocytopenia, anasarca, fever, renal impairment or reticulin fibrosis, and organomegaly (TAFRO) syndrome with unilateral adrenal necrosis: a case report","volume":"12","author":"Fujiwara","year":"2018","journal-title":"J Med Case Rep"},{"issue":"10","key":"2026040117232334200_luag006-B10","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.2169\/internalmedicine.56.7991","article-title":"Two cases of thrombocytopenia, anasarca, fever, reticulin fibrosis\/renal failure, and organomegaly (TAFRO) syndrome with high serum procalcitonin levels, including the first case complicated with adrenal hemorrhaging","volume":"56","author":"Nara","year":"2017","journal-title":"Intern Med"},{"issue":"5","key":"2026040117232334200_luag006-B11","doi-asserted-by":"crossref","first-page":"743","DOI":"10.2169\/internalmedicine.7976-21","article-title":"Bilateral adrenal infarctions as an initial manifestation of TAFRO syndrome: a case report and review of the literature","volume":"61","author":"Yonezaki","year":"2022","journal-title":"Intern Med"},{"key":"2026040117232334200_luag006-B12","doi-asserted-by":"crossref","first-page":"e919536","DOI":"10.12659\/AJCR.919536","article-title":"Thrombocytopenia, anasarca, fever, reticulin fibrosis\/renal failure, and organomegaly (TAFRO) syndrome with bilateral adrenal hemorrhage in two Caucasian patients","volume":"21","author":"Ducoux","year":"2020","journal-title":"Am J Case Rep"},{"issue":"2","key":"2026040117232334200_luag006-B13","doi-asserted-by":"crossref","first-page":"288","DOI":"10.2169\/naika.106.288","article-title":"TAFRO syndrome with bilateral adrenal hemorrhage","volume":"106","author":"Ito","year":"2017","journal-title":"Nihon Naika Gakkai Zasshi"},{"issue":"8","key":"2026040117232334200_luag006-B14","doi-asserted-by":"crossref","first-page":"1303","DOI":"10.2169\/internalmedicine.5850-20","article-title":"Complete resolution of a case of TAFRO syndrome accompanied by mediastinal panniculitis, adrenal lesion, and liver damage with hyperbilirubinemia","volume":"60","author":"Ono","year":"2021","journal-title":"Intern Med"},{"issue":"2","key":"2026040117232334200_luag006-B15","doi-asserted-by":"crossref","first-page":"742","DOI":"10.30574\/wjarr.2023.17.2.0300","article-title":"Peripheral hypothyroidism and TAFRO syndrome: particularity of 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