title: A life-threatening case of TAFRO syndrome with dramatic response to tocilizumab, rituximab, and pulse steroids: The first case report in Latin America.
abstract: BACKGROUND
  This is the report of the first case of TAFRO syndrome (Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly) in Latin America.
  
  
  METHODS
  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.
  
  
  METHODS
  Abdominal computed tomography (CT) showed bilateral pleural effusion and retroperitoneal lymph node enlargement.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Provided the interleukin-6 measurement is available, this approach is suggested in cases of TAFRO syndrome, in order to customize the treatment.
authors.affiliation: Department of Rheumatology Department of Hematology Department of Pathology, Hospital Israelita Albert Einstein Department of Pathology, Faculdade de Medicina da Universidade de São Paulo Department of Nuclear Medicine Department of Internal Medicine Department of Hematology, Hospital Israelita Albert Einstein, São Paulo, SP, Brazil.
authors.forename: Fabio Freire
authors.initials: FF
authors.lastname: José
authors.forename: Lucila Nassif
authors.initials: LN
authors.lastname: Kerbauy
authors.forename: Guilherme Fleury
authors.initials: GF
authors.lastname: Perini
authors.forename: Danielle Isadora
authors.initials: DI
authors.lastname: Blumenschein
authors.forename: Denise da Cunha
authors.initials: DDC
authors.lastname: Pasqualin
authors.forename: Denise Maria Avancini Costa
authors.initials: DMAC
authors.lastname: Malheiros
authors.forename: Guilherme de Carvalho
authors.initials: GC
authors.lastname: Campos Neto
authors.forename: Fabio Pires
authors.initials: FP
authors.lastname: de Souza Santos
authors.forename: Ronaldo
authors.initials: R
authors.lastname: Piovesan
authors.forename: Nelson
authors.initials: N
authors.lastname: Hamerschlak
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D005871:Castleman Disease / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage*; D008775:Methylprednisolone / Q000008:administration & dosage*; D008875:Middle Aged; D000069283:Rituximab / Q000008:administration & dosage*
chemical_list: D000893:Anti-Inflammatory Agents; D061067:Antibodies, Monoclonal, Humanized; D007155:Immunologic Factors; D000069283:Rituximab; C502936:tocilizumab; D008775:Methylprednisolone