TAFRO syndrome: 2 cases and review of the literature

Kawashima, Mikako; Usui, Toshio; Okada, Hironao; Mori, Ichiro; Yamauchi, Masahiro; Ikeda, Takahide; Kajita, Kazuo; Kito, Yusuke; Miyazaki, Tatsuhiko; Fujioka, Kei; Ishizuka, Tatsuo; Morita, Hiroyuki

doi:10.3109/14397595.2015.1059982

Cited by 32 publications

(28 citation statements)

References 16 publications

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“…In our case, the FDP and D-dimer levels were both increased, which might be due to pleural effusion and ascites. An elevation of the FDP and D-dimer levels has also been described in other case reports ( 3 - 6 , 8 , 10 - 15 ). We also speculate that these data may indicate the severity of TAFRO syndrome.…”

Section: Discussionsupporting

confidence: 76%

Successful Treatment of TAFRO Syndrome with Tocilizumab, Prednisone, and Cyclophosphamide

et al. 2017

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TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, pleural effusion, fever, renal dysfunction, reticulin fibrosis of the bone marrow, and organomegaly. The clinical course varies significantly among patients. However, the prognosis is usually dismal in patients with severe TAFRO syndrome, and no optimal treatment has yet been established. We herein describe the first case of TAFRO syndrome, which was successfully treated with combination therapy consisting of tocilizumab, prednisone, and cyclophosphamide.

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Section: Discussionsupporting

confidence: 76%

Successful Treatment of TAFRO Syndrome with Tocilizumab, Prednisone, and Cyclophosphamide

et al. 2017

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“…Eleven cases showed elevation of creatinine, 12 cases were positive for urinary protein or occult blood, and the renal biopsies in two cases showed mild to moderate proliferation of mesangial cells and thickened basement membrane with double contour and spike formation 15) .…”

Section: Discussionmentioning

confidence: 97%

Successful treatment by rituximab in a patient with TAFRO syndrome with cardiomyopathy

Hiramatsu¹,

Ohmura²,

Tsuji³

et al. 2016

Jpn. J. Clin. Immunol.

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TAFRO syndrome is a newly defined disease entity which is characterized by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. A histological pattern of multiple lymphadenopathy of atypical Castleman's disease (CD) is also an important characteristic. A 48-year-old man was referred to our hospital with fever, asthenia, bilateral pleural effusion, ascites, generalized edema, dyspnea, hypoalbuminemia, severe thrombocytopenia, anemia, renal failure and proteinuria, whereas bacterial culture and serological and PCR tests for various viruses were all negative. A CT scan showed multiple lymphadenopathy and tissue sampling of inguinal lymph nodes showed a compatible histology with plasma cell type CD. A diagnosis of TAFRO syndrome was made. Ten days after hospitalization, sudden cardiac insufficiency and anuria developed. Despite glucocorticoid pulse therapy, tocilizumab and plasmapheresis, clinical and laboratory features did not improve. On the 34(th) hospital day, we started rituximab. His general condition started to improve in several days, and by one month later anasarca had improved drastically. Thrombocytopenia and renal function gradually improved and finally normalized. Cardiac motion also improved. This is the first report of a TAFRO syndrome patient with cardiomyopathy, who was successfully treated with rituximab.

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“…Investigating renal pathology is important for understanding the pathophysiology of TAFRO syndrome. However, only a few case reports with kidney biopsy results have been published in the literature [49,53,55,56]. Performing kidney biopsy during the acute phase of TAFRO syndrome could be difficult because of the presence of massive ascites, presence of thrombocytopenia, and difficulty with placement in the prone position because of dyspnea.…”

Section: Discussionmentioning

confidence: 99%

Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

et al. 2018

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Background: TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal failure, and organomegaly) syndrome is a systemic inflammatory disorder and unique clinicopathological variant of idiopathic multicentric Castleman disease that was proposed in Japan. Prompt diagnosis is critical because TAFRO syndrome is a progressive and life threating disease. Some cases are refractory to immunosuppressive treatments. Renal impairment is frequently observed in patients with TAFRO syndrome, and some severe cases require hemodialysis. Histological evaluation is important to understand the pathophysiology of TAFRO syndrome. However, systemic histopathological evaluation through autopsy in TAFRO syndrome has been rarely reported previously. Case presentation: A 46-year-old Japanese man with chief complaints of fever and abdominal distension was diagnosed with TAFRO syndrome through imaging studies, laboratory findings, and pathological findings on cervical lymph node and bone marrow biopsies. Interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were remarkably elevated in both blood and ascites. Methylprednisolone (mPSL) pulse therapy was initiated on day 10, followed by combination therapy with PSL and cyclosporine A. However, the amount of ascites did not respond to the treatment. The patient became anuric, and continuous renal replacement therapy was initiated from day 50. However, the patient suddenly experienced cardiac arrest associated with myocardial infarction (MI) on the same day. Although the emergent percutaneous coronary intervention was successfully performed, the patient died on day 52, despite intensive care. Autopsy was performed to ascertain the cause of MI and to identify the histopathological characteristics of TAFRO syndrome.(Continued on next page)

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TAFRO syndrome: 2 cases and review of the literature

Cited by 32 publications

References 16 publications

Successful Treatment of TAFRO Syndrome with Tocilizumab, Prednisone, and Cyclophosphamide

Successful Treatment of TAFRO Syndrome with Tocilizumab, Prednisone, and Cyclophosphamide

Successful treatment by rituximab in a patient with TAFRO syndrome with cardiomyopathy

Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

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