TAFRO syndrome with refractory thrombocytopenia responding to tocilizumab and romiplostim: a case report

CEN Case Reports
Shoko Noda-NaritaYoshifumi Ubara

Abstract

Thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly (TAFRO) syndrome is a unique clinicopathologic subtype of multicentric Castleman's disease that has recently been identified in Japan. However, little is known about its renal histological changes and the optimal treatment for TAFRO syndrome. An 80-year-old Japanese woman was admitted to our hospital for evaluation of severe anasarca and weight gain (10 kg in a month). She had polyneuropathy, monoclonal plasma cell proliferative disorder with positive kappa M-protein, a sclerotic bone lesion, elevation of vascular endothelial growth factor (VEGF), skin changes, and extravascular volume overload, which fulfilled the diagnostic criteria for POEMS (polyneuropathy, organomegaly, endocrinopathy, and monoclonal protein, skin changes) syndrome. However, kappa-type M-protein and thrombocytopenia with positivity of platelet-associated immunoglobulin G antibody were unusual, and fitted the diagnostic criteria for TAFRO syndrome. Renal biopsy showed diffuse endocapillary proliferative glomerulonephritis with endothelial swelling and the infiltration of monocytes and neutrophils without specific immunoglobulin deposits. Her systemic symptoms were refractory to initial trea...Continue Reading

References

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Citations

Jun 23, 2020·Pediatric Blood & Cancer·Shogo MinamikawaRyojiro Tanaka
May 12, 2019·CEN Case Reports·Hirotaka SaitoJunichiro James Kazama
Oct 19, 2019·BMC Nephrology·Yoshikuni NagayamaKatsuyuki Matsui
Dec 18, 2020·Internal Medicine·Sho ShibataToshiyuki Kitano
Oct 23, 2020·Modern Rheumatology Case Reports·Yuta YamaguchiAtsushi Kumanogoh
Feb 20, 2021·Clinical and Experimental Nephrology·Yoshifumi UbaraUNKNOWN Committee of Practical Guide for Kidney Biopsy 2020

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