Adolescent-onset TAFRO Syndrome with Malignant Nephrosclerosis-like Lesions

A 16-year-old Japanese girl developed a fever, thrombocytopenia, and renal dysfunction. Treatment was started with steroids, but cervical lymphadenopathy and ascites developed. A lymph node biopsy indicated TAFRO syndrome. The patient's renal function deteriorated, and dialysis was started. Ref...

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Detalles Bibliográficos
Autores principales: Nakayama, Yuki, Mizuno, Hiroki, Sawa, Naoki, Suwabe, Tatsuya, Yamanouchi, Masayuki, Ikuma, Daisuke, Hasegawa, Eiko, Hoshino, Junichi, Sekine, Akinari, Oba, Yuki, Kono, Kei, Kinowaki, Keiichi, Ohashi, Kenichi, Suzuki, Kodai, Sato, Yasuharu, Shimizu, Akira, Yamaguchi, Yutaka, Ubara, Yoshifumi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10465276/
https://www.ncbi.nlm.nih.gov/pubmed/36517029
http://dx.doi.org/10.2169/internalmedicine.0529-22
Descripción
Sumario:A 16-year-old Japanese girl developed a fever, thrombocytopenia, and renal dysfunction. Treatment was started with steroids, but cervical lymphadenopathy and ascites developed. A lymph node biopsy indicated TAFRO syndrome. The patient's renal function deteriorated, and dialysis was started. Refractory hypertension and subsequent encephalopathy developed. Treatment was started with an anti-IL-6 receptor antibody and an anti-CD20 monoclonal antibody. A kidney biopsy showed malignant nephrosclerosis-like microangiopathy and glomerular collapse due to narrowing of the small arteries. The majority of TAFRO syndrome cases are adult-onset, with glomerular microangiopathy. To our knowledge, this is the first report of adolescent-onset TAFRO syndrome presenting with malignant nephrosclerosis-like lesions associated with hypertension.

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