{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:48:08Z","timestamp":1760147288247,"version":"build-2065373602"},"reference-count":21,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2023,1,23]],"date-time":"2023-01-23T00:00:00Z","timestamp":1674432000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Histopathologic findings in the lymph nodes of patients with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome are similar to those of idiopathic multicentric Castleman\u2019s disease (iMCD), but TAFRO syndrome is different from iMCD in how it can progress rapidly and be fatal. These patients present scarce lymphadenopathy and low immunoglobulin levels. We present a case of cutaneous and systemic plasmacytosis (C\/SP) that caused TAFRO syndrome-like symptoms which were successfully treated with rituximab. A 67-year-old woman presented with fever and a pruritic skin rash. Numerous plasma cells were observed in the peripheral blood and imaging revealed organomegaly, anasarca, and generalized lymphadenopathy. Subsequently, she rapidly developed thrombocytopenia as well as renal and heart failure. She tested positive for the Epstein\u2013Barr virus (EBV), elevated immunoglobulins, and C\/SP, which are also atypical for TAFRO syndrome, thereby complicating the diagnosis. However, after using the Japanese TAFRO Syndrome Research Group diagnostic criteria, we promptly administered rituximab to treat the C\/SP with TAFRO-like symptoms and saved her life. Finally, histopathological observations of the lymph node biopsy helped confirm EBV-positive hypervascular-type iMCD. Therefore, diagnosing TAFRO-like syndromes based on the Japanese diagnostic criteria and following the associated treatment even without a confirmed diagnosis is crucial to improving the patient outcomes.<\/jats:p>","DOI":"10.3390\/medicina59020216","type":"journal-article","created":{"date-parts":[[2023,1,23]],"date-time":"2023-01-23T02:27:45Z","timestamp":1674440865000},"page":"216","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Epstein\u2013Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab"],"prefix":"10.3390","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2065-1957","authenticated-orcid":false,"given":"Seiji","family":"Kakiuchi","sequence":"first","affiliation":[{"name":"Department of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroaki","family":"Akiyama","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isamu","family":"Harima","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ikumi","family":"Takagi","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junpei","family":"Rikitake","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoko","family":"Kozuki","sequence":"additional","affiliation":[{"name":"Department of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mayumi","family":"Inaba","sequence":"additional","affiliation":[{"name":"Department of Pathology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Fujiwara","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nozomu","family":"Kurose","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1, Daigaku, Uchinada, Kahoku 920-0293, Ishikawa, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2662-0024","authenticated-orcid":false,"given":"Sohsuke","family":"Yamada","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1, Daigaku, Uchinada, Kahoku 920-0293, Ishikawa, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5658-3765","authenticated-orcid":false,"given":"Yasufumi","family":"Masaki","sequence":"additional","affiliation":[{"name":"Department of Hematology and Immunology, Kanazawa Medical University, 1-1, Daigaku, Uchinada, Kahoku 920-0293, Ishikawa, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,1,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e236283","DOI":"10.1136\/bcr-2020-236283","article-title":"Idiopathic multicentric Castleman disease preceded by cutaneous plasmacytosis successfully treated by tocilizumab","volume":"13","author":"Aita","year":"2020","journal-title":"BMJ Case Rep."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1097\/DAD.0000000000002109","article-title":"Cutaneous plasmacytosis and idiopathic multicentric Castleman disease: A spectrum of disease?","volume":"44","author":"Drissi","year":"2022","journal-title":"Am. 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