Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version

Abstract

TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca including pleural effusion and ascites, fever, renal insufficiency, and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology is undetermined. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of multicentric Castleman disease (MCD), other specific features can differentiate between them. Some TAFRO syndrome patients have been successfully treated with glucocorticoids and/or immunosuppressants, including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, and eventually succumb to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The present article reports the 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams. These criteria and classification have been applied and retrospectively validated on clinicopathologic data of 28 patients with this and similar conditions (e.g. MCD with serositis and thrombocytopenia).

Cardiac arrest caused by rapidly increasing ascites in a patient with TAFRO syndrome: a case report

“…In some reports, the cause of death was multiple organ failure secondary to deteriorating TAFRO syndrome over a 2‐month period and septic shock associated with febrile neutropenia during an 8‐month observation period 2. Although no reports have described acute life‐threatening symptoms in patients with TAFRO syndrome, we assume that TAFRO syndrome may cause death in the acute phase.…”

“…The mortality rate is 11–12% 2, 5. In some reports, the cause of death was multiple organ failure secondary to deteriorating TAFRO syndrome over a 2‐month period and septic shock associated with febrile neutropenia during an 8‐month observation period 2.…”

“…The diagnostic criteria for TAFRO syndrome were announced by two groups 2, 5. Both these criteria emphasize clinical findings, however, one requires the histological feature in lymph node, the other does not.…”

“…Thrombocytopenia, anasarca (including pleural fluid and ascites), fever, renal insufficiency, and organomegaly (TAFRO) syndrome is a systemic inflammatory disorder first reported in 2010 1, 2. This syndrome is considered a variant of multicentric Castleman's disease, but its pathogenetic mechanism remains unknown.…”

“…The diagnostic criteria, severity classification, and treatment strategy were recently announced by a Japanese group (Table 1). 2 The mortality rate is 11–12%, and death often occurs after a relatively long disease course 2, 5. The main causes of death are multiple organ dysfunction unresponsive to immunosuppressive agents and severe infection caused by an immunocompromised state.…”