Complete Resolution of TAFRO Syndrome (Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis and Organomegaly) after Immunosuppressive Therapies using Corticosteroids and Cyclosporin A : A Case Report

Inoue, Morihiro; Ankou, Mayuka; Hua, Jiang; Iwaki, Yasunobu; Hagihara, Masao; Ota, Yasunori

doi:10.3960/jslrt.53.95

Cited by 68 publications

(57 citation statements)

References 17 publications

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“…As in our case, some patients with TAFRO syndrome have been reported to achieve remission with CyA treatment (Takai et al 2010;Inoue et al 2013). However, in contrast to our patient, patients in previous reports did not receive tocilizumab therapy.…”

Section: Discussioncontrasting

confidence: 54%

“…Takai et al (2010) reported three patients who shared a constellation of symptoms, thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly, as well as hyaline-vascular-type histology in the lymph node of one patient. Since then, additional case reports (Inoue et al 2013;Masaki et al 2013) involving this constellation of symptoms have been published. These findings collectively suggest a novel clinical entity comprising systemic inflammatory disorders on a background of immunological abnormality that exceeds the ordinary spectrum of MCD.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussioncontrasting

confidence: 54%

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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“…83,84 Immunosuppressive therapies, particularly cyclosporine A, are being used more frequently as some physicians are treating iMCD more like a systemic inflammatory disease. 85,86 Rituximab, which is a frequent first-or second-line therapy in iMCD, is only partially effective and typically does not provide long-term disease control. [87][88][89] Cytotoxic lymphoma-based chemotherapies (eg, cyclophosphamide, doxorubicin, vincristine, and prednisone) induce responses in a large portion of the most severely ill iMCD patients by eliminating a large portion of hypercytokine-secreting cells, but relapses are common and side effects are significant.…”

Section: Treatmentmentioning

confidence: 99%

HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy

Fajgenbaum

Rhee

Nabel

2014

Blood

280

265

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Multicentric Castleman's disease (MCD) describes a heterogeneous group of disorders involving proliferation of morphologically benign lymphocytes due to excessive proinflammatory hypercytokinemia, most notably of interleukin-6. Patients demonstrate intense episodes of systemic inflammatory symptoms, polyclonal lymphocyte and plasma cell proliferation, autoimmune manifestations, and organ system impairment. Human herpes virus-8 (HHV-8) drives the hypercytokinemia in all HIV-positive patients and some HIV-negative patients. There is also a group of HIV-negative and HHV-8-negative patients with unknown etiology and pathophysiology, which we propose referring to as idiopathic MCD (iMCD). Here, we synthesize what is known about iMCD pathogenesis, present a new subclassification system, and propose a model of iMCD pathogenesis. MCD should be subdivided into HHV-8-associated MCD and HHV-8-negative MCD or iMCD. The lymphocyte proliferation, histopathology, and systemic features in iMCD are secondary to hypercytokinemia, which can occur with several other diseases. We propose that 1 or more of the following 3 candidate processes may drive iMCD hypercytokinemia: systemic inflammatory disease mechanisms via autoantibodies or inflammatory gene mutations, paraneoplastic syndrome mechanisms via ectopic cytokine secretion, and/or a non-HHV-8 virus. Urgent priorities include elucidating the process driving iMCD hypercytokinemia, identifying the hypercytokine-secreting cell, developing consensus criteria for diagnosis, and building a patient registry to track cases.

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“…11,14,[16][17][18][19][20] iMCD patients without TAFRO syndrome typically have thrombocytosis, hypergammaglobulinemia, and less severe fluid accumulation. This non-TAFRO group has been called idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia or IPL-type.…”

Section: Introductionmentioning

confidence: 99%

International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease

Fajgenbaum

Uldrick

Bagg

et al. 2017

Blood

460

640

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Complete Resolution of TAFRO Syndrome (Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis and Organomegaly) after Immunosuppressive Therapies using Corticosteroids and Cyclosporin A : A Case Report

Cited by 68 publications

References 17 publications

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

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

HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy

International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease

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