Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab
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’s disease (iMCD), but TAFRO syndrome is different from iMCD in how it can...
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MDPI AG
2023-01-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/59/2/216 |
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| author | Seiji Kakiuchi Hiroaki Akiyama Isamu Harima Ikumi Takagi Junpei Rikitake Yoko Kozuki Mayumi Inaba Hiroshi Fujiwara Nozomu Kurose Sohsuke Yamada Yasufumi Masaki |
| author_facet | Seiji Kakiuchi Hiroaki Akiyama Isamu Harima Ikumi Takagi Junpei Rikitake Yoko Kozuki Mayumi Inaba Hiroshi Fujiwara Nozomu Kurose Sohsuke Yamada Yasufumi Masaki |
| author_sort | Seiji Kakiuchi |
| collection | DOAJ |
| description | 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’s 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–Barr 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. |
| first_indexed | 2024-03-11T08:27:07Z |
| format | Article |
| id | doaj.art-3052587927d74d0786caa13d0bdc5f32 |
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| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-11T08:27:07Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-3052587927d74d0786caa13d0bdc5f322023-11-16T21:58:43ZengMDPI AGMedicina1010-660X1648-91442023-01-0159221610.3390/medicina59020216Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with RituximabSeiji Kakiuchi0Hiroaki Akiyama1Isamu Harima2Ikumi Takagi3Junpei Rikitake4Yoko Kozuki5Mayumi Inaba6Hiroshi Fujiwara7Nozomu Kurose8Sohsuke Yamada9Yasufumi Masaki10Department of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Hematology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Pathology, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Respiratory Medicine, Yodogawa Christian Hospital, 1-7-50, Kunijima, Higashi-Yodogawa-ku, Osaka 533-0024, JapanDepartment of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1, Daigaku, Uchinada, Kahoku 920-0293, Ishikawa, JapanDepartment of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1, Daigaku, Uchinada, Kahoku 920-0293, Ishikawa, JapanDepartment of Hematology and Immunology, Kanazawa Medical University, 1-1, Daigaku, Uchinada, Kahoku 920-0293, Ishikawa, JapanHistopathologic 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’s 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–Barr 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.https://www.mdpi.com/1648-9144/59/2/216TAFRO syndromeidiopathic multicentric Castleman’s diseasecutaneous and systemic plasmacytosisEpstein–Barr virusrituximab |
| spellingShingle | Seiji Kakiuchi Hiroaki Akiyama Isamu Harima Ikumi Takagi Junpei Rikitake Yoko Kozuki Mayumi Inaba Hiroshi Fujiwara Nozomu Kurose Sohsuke Yamada Yasufumi Masaki Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab Medicina TAFRO syndrome idiopathic multicentric Castleman’s disease cutaneous and systemic plasmacytosis Epstein–Barr virus rituximab |
| title | Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab |
| title_full | Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab |
| title_fullStr | Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab |
| title_full_unstemmed | Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab |
| title_short | Epstein–Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab |
| title_sort | epstein barr virus positive cutaneous and systemic plasmacytosis with tafro syndrome like symptoms successfully treated with rituximab |
| topic | TAFRO syndrome idiopathic multicentric Castleman’s disease cutaneous and systemic plasmacytosis Epstein–Barr virus rituximab |
| url | https://www.mdpi.com/1648-9144/59/2/216 |
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