Castleman-Kojima Disease in a South Asian Adolescent

Koduri, Prasad Rao; Mohammed, Parvez; Kaza, Sashidhar; Pappu, Prabhakar; Sekaran, Anuradha

doi:10.3960/jslrt.54.163

Cited by 17 publications

(12 citation statements)

References 14 publications

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“…Ideally chemotherapy would provide a means to eliminate large portions of hypercytokine-secreting cells; however, its use without immunosuppressive therapy has not had lasting and meaningful results [ 12 , 15 ]. Only one patient in the literature [ 16 ] had been treated with regimen consistent of rituximab, tocilizumab, cyclophosphamide, and etoposide.…”

Section: Discussionmentioning

confidence: 99%

TAFRO Syndrome Associated with EBV and Successful Triple Therapy Treatment: Case Report and Review of the Literature

Simons

Apor

Butera

et al. 2016

Case Reports in Hematology

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TAFRO syndrome is a rare constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction, and organomegaly. Its pathogenesis involves an excessive and inappropriate cytokine storm, most notably from IL-6, causing multiorgan failure; however, its etiology is undetermined. Starting in 2012, TAFRO syndrome was first identified in Japan as an atypical variant of Castleman's disease. Previous reports include various different treatment protocols with inconsistent survival outcomes. Here we report the first known American, EBV positive but HIV and HHV-8 negative, male with TAFRO syndrome. He was successfully treated with an unusual three-drug regimen including tocilizumab, etoposide, and rituximab. We review the literature of TAFRO syndrome, discuss its possible viral etiology, and propose an original treatment regimen.

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Section: Discussionmentioning

confidence: 99%

TAFRO Syndrome Associated with EBV and Successful Triple Therapy Treatment: Case Report and Review of the Literature

Simons

Apor

Butera

et al. 2016

Case Reports in Hematology

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“…10 Therefore, it was considered difficult to predict treatment resistance to tocilizumab by serum IL-6 alone. 10 Our patient was a very rare pediatric case with TAFRO syndrome, [21][22][23][24][25] and the first reported pediatric case showing tocilizumab resistance. Pediatricians need to be aware of TAFRO syndrome, because the number of cases with onset at a young age is increasing.…”

Section: A Pediatric Case Of Tocilizumab-resistant Tafro Syndrome Trementioning

confidence: 99%

A pediatric case of tocilizumab‐resistant TAFRO syndrome treated successfully with rituximab

Minamikawa

Matsumura

Sato

et al. 2020

Pediatric Blood & Cancer

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“…Accordingly, Kawabata et al (2013) proposed the clinical term "TAFRO" (i.e., thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly) syndrome, for which the proposed diagnostic criteria are shown in Table 1. However, a case of a South Asian adolescent with TAFRO syndrome was recently reported in India (Koduri et al 2014), indicating that TAFRO syndrome is not endemic only to Japan. The pathophysiology of TAFRO syndrome is poorly understood, and no therapeutic strategy has been established.…”

Section: Introductionmentioning

confidence: 99%

Successful Treatment of TAFRO Syndrome, a Variant of Multicentric Castleman’s Disease, with Cyclosporine A: Possible Pathogenetic Contribution of Interleukin-2

Konishi

Takahashi

Nishi

et al. 2015

Tohoku J. Exp. Med.

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Multicentric Castleman's disease is a systemic inflammatory disorder characterized by lymphadenopathy and excessive interleukin-6 production. A unique clinicopathologic variant of multicentric Castleman's disease, TAFRO (i.e., thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly) syndrome, was recently proposed in Japan. Despite the successful use of anti-interleukin-6 therapy in some patients with TAFRO syndrome, not all patients achieve remission. The pathophysiological etiology of and suitable therapeutic strategies for this variant have not been established. Here, we present our experience of a unique case of TAFRO syndrome in a 78-year-old woman whose symptoms responded differently to several therapies. Tocilizumab, an anti-interleukin-6 receptor antibody, successfully induced remission of fever and lymphadenopathy. However, severe thrombocytopenia persisted and she developed anasarca, ascites, and pleural effusion shortly thereafter. Rituximab, an anti-CD20 antibody, and glucocorticoid therapy provided no symptom relief. In contrast, cyclosporine A, an immunosuppressive agent that blocks T cell function by inhibiting interleukin-2, yielded immediate improvements in systemic fluid retention and a gradual increase in platelet count, with complete resolution of disease symptoms. Excessive serum interleukin-2, when used as an anti-cancer agent, has been reported to cause side effects such as fluid retention, thrombocytopenia, and renal failure. Our case was unique because the antiinterleukin-2 therapy successfully improved symptoms that were not relieved with anti-interleukin-6 therapy. The present report therefore provides insight into the possible role of interleukin-2, in addition to interleukin-6, in TAFRO syndrome. This report will certainly help to clarify the pathogenesis of and optimal treatment strategies for TAFRO syndrome.

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Castleman-Kojima Disease in a South Asian Adolescent

Cited by 17 publications

References 14 publications

TAFRO Syndrome Associated with EBV and Successful Triple Therapy Treatment: Case Report and Review of the Literature

TAFRO Syndrome Associated with EBV and Successful Triple Therapy Treatment: Case Report and Review of the Literature

A pediatric case of tocilizumab‐resistant TAFRO syndrome treated successfully with rituximab

Successful Treatment of TAFRO Syndrome, a Variant of Multicentric Castleman’s Disease, with Cyclosporine A: Possible Pathogenetic Contribution of Interleukin-2

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