Renal involvement in TAFRO syndrome: a review

Area of Science:

• Nephrology
• Pathology
• Hematology

Background:

• TAFRO syndrome is a rare hematologic disorder.
• Renal involvement is a critical clinical feature.
• Understanding kidney pathology is crucial for diagnosis and management.

Purpose of the Study:

• To detail the clinicopathologic features of renal involvement in TAFRO syndrome.
• To differentiate renal pathology between adult and adolescent patients.
• To highlight diagnostic markers for TAFRO syndrome-associated kidney disease.

Main Methods:

• Clinical data review including renal function and proteinuria.
• Histopathological analysis of kidney biopsies (light microscopy, immunofluorescence, electron microscopy).
• Comparison of findings between adult and adolescent cohorts.

Main Results:

• Renal involvement presents with edema, ascites, effusions, and rapid renal function decline (<1 g/day proteinuria).
• Histology reveals glomerular microangiopathy (mesangiolysis, endothelial proliferation, GBM doubling), without significant immunodeposits or thrombi.
• Adolescents exhibit additional arteriolar intimal proliferation and vascular occlusion, distinguishing them from adults.

Conclusions:

• TAFRO syndrome causes a unique glomerular microangiopathy.
• Adolescent TAFRO syndrome shows distinct vascular lesions.
• These findings aid in diagnosing and understanding TAFRO syndrome's renal manifestations.