A life-threatening case of TAFRO syndrome with dramatic response to tocilizumab, rituximab, and pulse steroids: The first case report in Latin America.

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.

• Authors: Fabio Freire José, Lucila Nassif Kerbauy, Guilherme Fleury Perini, Danielle Isadora Blumenschein, Denise da Cunha Pasqualin, Denise Maria Avancini Costa Malheiros, Guilherme de Carvalho Campos Neto, Fabio Pires de Souza Santos, Ronaldo Piovesan, Nelson Hamerschlak
• Journal: Medicine (Medicine (Baltimore)) Vol. 96 Issue 13 Pg. e6271 (Mar 2017) ISSN: 1536-5964 [Electronic] United States
• PMID: 28353560 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anti-Inflammatory Agents
• Antibodies, Monoclonal, Humanized
• Immunologic Factors
• Rituximab
• tocilizumab
• Methylprednisolone

Topics

• Anti-Inflammatory Agents (administration & dosage)
• Antibodies, Monoclonal, Humanized (administration & dosage)
• Castleman Disease (drug therapy)
• Drug Therapy, Combination
• Female
• Humans
• Immunologic Factors (administration & dosage)
• Methylprednisolone (administration & dosage)
• Middle Aged
• Rituximab (administration & dosage)