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 treatment with high-dose
melphalan and
glucocorticoids. Alternative
therapy with an anti-interleukin-6 (IL-6) receptor antibody (
tocilizumab) effectively controlled the symptoms, while a
thrombopoietin receptor agonist (
romiplostim) was effective for her
thrombocytopenia. Results suggest that IL-6-VEGF axis and an autoimmune mechanism may be responsible for
TAFRO syndrome with clinical features of POEMS and refractory
thrombocytopenia, which can be successfully treated with combination of
tocilizumab and
romiplostim.