TAFRO syndrome presenting with lymphoma suspicion: Diagnostic challenge and rapid clinical course in a young patient

Abstract

A 29-year-old female patient presented with abdominal pain and edema. Imaging revealed cervical, axillary, and abdominal multiple lymphadenopathies, splenomegaly, and massive ascites. An axillary lymph node biopsy performed at an outside center was reported as “suspicious for T-cell lymphoma, further evaluation at a reference center recommended.” PET-CT showed lymphadenopathies with SUVmax not exceeding 5. The biopsy was re-evaluated at our hospital and interpreted as reactive; no characteristic Castleman-type morphology was reported. During follow-up, acute kidney injury developed and the patient required hemodialysis. Massive pleural and pericardial effusions and ascites were detected simultaneously. Cytological examination of the ascitic and pleural fluids was negative for malignancy. Peak C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were 184 mg/L and 110 mm/h, respectively. Hemoglobin was low, platelet count progressively decreased, and bone marrow biopsy revealed reticulin fibrosis involving approximately 10% of the sampled marrow. A diagnosis of TAFRO syndrome was made, and an off-label application for tocilizumab was submitted; however, the patient succumbed to septic shock before tocilizumab could be administered. This case highlights that although rare, TAFRO should be considered in the differential diagnosis and can follow an aggressive course.

Keywords

• TAFRO syndrome
• Multicentric Castleman disease
• Lymphoma mimicry

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