A life-threatening case of TAFRO syndrome with dramatic response to tocilizumab, rituximab, and pulse steroids

Abstract

Rationale:This is the report of the first case of TAFRO syndrome (Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly) in Latin America.Patient concerns:The patient was a 61-year-old white woman of Ashkenazi Jewish descent, who presented with a history of 8 days of nausea, vomiting, and fever; severe pitting edema in both legs, ascites, splenomegaly, and palpable axillary lymph nodes.Diagnoses:Abdominal computed tomography (CT) showed bilateral pleural effusion and retroperitoneal lymph node enlargement.Interventions:Anasarca and worsening of renal function led to admission to the intensive care unit (ICU) with multiple organ failure, requiring mechanical ventilation, vasopressor medications, and continuous renal replacement therapy (CRRT). Diagnosis of TAFRO syndrome was made on day 18 after admission, based on clinical findings and results of bone marrow and lymph node biopsies. She was treated with methylprednisolone, tocilizumab, and rituximab. One week after the first tocilizumab dose, she had dramatic improvements in respiratory and hemodynamic status, and was weaned from ventilator support and vasopressor medications.Outcomes:After 2 weeks of therapy, CRRT was switched to intermittent hemodialysis. On day 46, the patient was discharged from the ICU to the general ward, and 3 months after admission, she went home.Lessons:Provided the interleukin-6 measurement is available, this approach is suggested in cases of TAFRO syndrome, in order to customize the treatment.

TAFRO Syndrome in Caucasians: A Case Report and Review of the Literature

“…In comparison with the Japanese cohort, of 18 patients we find Caucasian TAFRO cases presented more frequently with kidney failure and auto-immune antibodies, although these results need to be interpreted with caution due to the low number of patients (Table 1 ). Recently, a patient with TAFRO was also reported in Latin America ( 21 ) and the members of the Castleman Disease Collaborative Network report treating iMCD patients with TAFRO from diverse ethnic backgrounds throughout the world for decades ( 11 ). As to IgG4-related disease primary reported in Japan ( 22 ), TAFRO syndrome is a rare entity affecting patients with different ethnic background without major differences in the clinicopathological presentation.…”

Comparison of the clinical characteristics of TAFRO syndrome and idiopathic multicentric Castleman disease in general internal medicine: a 6‐year retrospective study

“…8 Previously, MCD was considered to occur mainly in association with a human herpes virus 8 (HHV-8) infection in immunocompromised patients. [10][11][12][13] Although the clinical characteristics are similar in these two disorders, TAFRO syndrome has been described as a clinical entity distinct from iMCD because TAFRO syndrome is usually associated with hypogammaglobulinaemia, which is in stark contrast to iMCD, and with poor treatment response. 7 iMCD was thus further defined by HIV-and HHV-8-negative MCD subtypes.…”

Frontline Siltuximab and Rituximab in TAFRO syndrome: A case report

“…The anti‐IL6 agent siltuximab has been recently FDA and EMA approved for frontline use in critically ill patients with multicenter Castleman's disease 4 . A higher chance of response to siltuximab was reported in patients with higher fibrinogen, hemoglobin and IgG serum levels, 5 and frontline association between anti‐IL6 agents and rituximab had been previously reported for severe cases 6,7 ; therefore, this patient continued rituximab infusions (375 mg per squared meter every 3 weeks) but also started siltuximab 11 mg/kg every 3 weeks (after two weekly loading doses). In a 2‐week time, the patient anasarca rapidly ameliorated, unconsciousness resolved, amine support could be stopped and axillary lymph nodes vanished as well as splenomegaly.…”