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.

Figure 1

Histopathological examination of the lymph node, with immunohistochemical evaluation. (A) Lymph node with involuted and hyalinized germinal center. Vascular proliferation of the interfollicular area. Hematoxylin and eosin stain, 100x magnification. (B) CD138 stain (MI15, Dako) of lymph node showing section of lymph node with involuted hyalinized germinal centers and interfollicular areas with vascular hyperplasia and increased scattered plasma cells. 100x magnification. (C) Renal glomerulus with turgescence of endothelial cells, mesangiolysis and subendothelial space widening, and clarification. Masson trichrome stain, 200x magnification. (D) Renal glomerulus with subendothelial mesangial interposition (double contours) of glomerular basement membranes, simulating a membranoproliferative pattern; however, no immune complexes were present. PAS stain, 200x.

Figure 2

Positron-emission tomography (PET-CT) scan. A. PET-CT fusion image before the start of therapy at the thoracic level, showing fluorodeoxyglucose (FDG) uptake in enlarged axillary and mediastinal lymph nodes and the presence of bilateral pleural effusion. (B) PET-CT after one month of therapy showing reduction in lymph node size and FDG uptake and resolution of the pleural effusion.