To and TAFRO - a cryptic cause of acute renal failure: a case report.

ABSTRACT

BACKGROUND: TAFRO syndrome is a rare clinical subtype of idiopathic multicentric Castlemans disease characterised by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Renal involvement is common, sometimes requiring temporary renal replacement therapy. Due to the associated thrombocytopenia, renal biopsies are rarely performed limiting descriptions of the renal histopathology in this condition. This case describes a patient with TAFRO syndrome and the associated renal histology. CASE PRESENTATION A 49-year-old Caucasian man presented to a tertiary hospital in Sydney with a six- week history of malaise, non-bloody diarrhoea, progressive shortness of breath, and drenching night sweats. A progressive bicytopenia and renal function decline necessitating temporary dialysis prompted a bone marrow aspirate and trephine, as well as a renal biopsy respectively. This noted a hypercellular bone marrow with increased granulopoiesis, reduced erythropoiesis, and fibrosis, with renal histology suggesting a thrombotic microangiopathic-like glomerulopathy. Alternate conditions were excluded, and a diagnosis of TAFRO syndrome was made. Glucocorticoids and rituximab were initiated with rapid renal recovery, and normalisation of his haematologic parameters achieved at six months. CONCLUSION: This case describes an atypical thrombotic microangiopathy as the predominant histologic renal lesion in a patient with TAFRO syndrome. This was responsive to immunosuppression with glucocorticoids and rituximab, highlighting the importance of early recognition of this rarely described condition.