Epstein-Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab

• Published in: Medicina (Kaunas, Lithuania) Lithuania), 2023-01, Vol.59 (2), p.216
• Main Authors: Kakiuchi, Seiji, Akiyama, Hiroaki, Harima, Isamu, Takagi, Ikumi, Rikitake, Junpei, Kozuki, Yoko, Inaba, Mayumi, Fujiwara, Hiroshi, Kurose, Nozomu, Yamada, Sohsuke, Masaki, Yasufumi
• Format: Article
• Language: English
• Subjects: Aged; Analysis; Biopsy; Blood pressure; Blood tests; Bone marrow; Case Report; cutaneous and systemic plasmacytosis; Cytokines; Dendritic cells; Drug therapy; Edema; Epstein-Barr virus; Epstein-Barr Virus Infections - complications; Erythema; Female; Fever; Herpesvirus 4, Human; Histology, Pathological; Histopathology; Humans; idiopathic multicentric Castleman’s disease; Immunoglobulins; Immunotherapy; Infections; Lymphadenopathy - complications; Lymphatic diseases; Lymphatic system; Monoclonal antibodies; Myocarditis; Patient outcomes; Plasma; Pruritus; Rituximab; Steroids; TAFRO syndrome; Thrombocytopenia; Thrombocytopenia - complications; Thrombocytopenia - diagnosis; Thrombocytopenia - pathology
• ISSN: 1648-9144; 1010-660X; 1648-9144
• DOI: 10.3390/medicina59020216

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.