Renal histology in a patient with TAFRO Syndrome: A case report

Publication date: Available online 4 April 2018
Source:Human Pathology
Author(s): Hiroki Mizuno, Akinari Sekine, Masahiko Oguro, Yoichi Oshima, Masahiro Kawada, Keiichi Sumida, Masayuki Yamanouchi, Noriko Hayami, Tatsuya Suwabe, Rikako Hiramatsu, Eiko Hasegawa, Junichi Hoshino, Naoki Sawa, Takashi Fujii, Kenmei Takaichi, Kenichi Ohashi, Yoshifumi Ubara
An 84-year-old Japanese man was admitted due to anasarca, thrombocytopenia, systemic inflammation, and progressive renal insufficiency, resistant to diuretics, glucocorticoid therapy and plasma exchange. Renal biopsy showed diffuse endocapillary proliferation and mesangiolysis without any immune deposits. Tocilizumab suppressed systemic inflammation, resulting in improvement of anasarca and renal dysfunction, but thrombocytopenia persisted and platelet-associated IgG antibody was elevated. Though romiplostim was effective for thrombocytopenia, the patient died of aspiration pneumonia after cerebral hemorrhage. Autopsy showed the hyaline vascular type Castleman's disease-like lymphadenopathy and reticulin myelofibrosis with an increase of megakaryocytes. Renal finding showed that endocapillary injury improved, and collapsed glomeruli was noted. This patient fitted the criteria of TAFRO (thrombocytopenia (T), anasarca (A), fever (F), reticulin myelofibrosis (R), and organomegaly (O)) syndrome. The clinical course suggests that two factors, including overproduction of interleukin-6 and autoimmune-mediated thrombocytopenia via thrombopoietin receptor, may have contributed to the pathogenesis of TAFRO syndrome in this patient.



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