Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 6, 2020; 8(9): 1656-1665
Published online May 6, 2020. doi: 10.12998/wjcc.v8.i9.1656
Liver cirrhosis in a child associated with Castleman's disease: A case report
Soya Kobayashi, Ayano Inui, Tomoyuki Tsunoda, Syuichiro Umetsu, Tsuyoshi Sogo, Masaaki Mori, Masato Shinkai, Tomoo Fujisawa
Soya Kobayashi, Ayano Inui, Tomoyuki Tsunoda, Syuichiro Umetsu, Tsuyoshi Sogo, Tomoo Fujisawa, Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohama City Tobu Hospital, Yokohama 230-8765, Japan
Masaaki Mori, Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo 113-8519, Japan
Masato Shinkai, Department of Surgery, Kanagawa Children’s Medical Center, Yokohama 232-8555, Japan
Author contributions: Kobayashi S and Inui A designed the report; Kobayashi S collected the patient’s clinical data; Tsunoda T, Umetsu S, Sogo T, Mori M and Fujisawa T participated in study design; Kobayashi S and Inui A analyzed the data and wrote the paper; Shinkai M performed a lymph node biopsy; all authors have finally approved the version of the article to be published.
Informed consent statement: Informed written consent was obtained from the patient and his parents for publication of this report and any accompanying images.
Conflict-of-interest statement: The fifth author accepts remuneration from "EA Pharma Co., Ltd., Otsuka Pharmaceutical Factory, Inc." The sixth author accepts remuneration from "Chugai Pharmaceutical Co., Ltd., UCB Japan Co., Ltd., CSL Behring, Abbvie Japan Co., Ltd., Japan Blood Products Organization, Ayumi Pharmaceutical Co., Nippon Kayaku Co., Ltd., MSD K.K., Daiichi Sankyo Co., Ltd., Taisho Pharmaceutical Co., Ltd., and Asahikasei Pharmaceutical Co." Other authors declare that there is no conflict of interest. Even though there is a COI to disclose as of January 2020, this will not prevent the submission from proceeding.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Ayano Inui, MD, PhD, Director, Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohama City Tobu Hospital, 3-6-1 Shimosueyoshi, Tsurumi-ku, Yokohama 230-8765, Japan. a_inui@tobu.saiseikai.or.jp
Received: January 30, 2020
Peer-review started: January 30, 2020
First decision: March 18, 2020
Revised: March 27, 2020
Accepted: April 21, 2020
Article in press: April 21, 2020
Published online: May 6, 2020
Processing time: 91 Days and 5.8 Hours
Abstract
BACKGROUND

Castleman's disease (CD) is a lymphoproliferative disorder. TAFRO syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia, anasarca (generalized edema and pleural effusion), fever (pyrexia), reticulin fibrosis in the bone marrow and the proliferation of megakaryocytes, and organomegaly (such as hepatosplenomegaly and multiple lymphadenopathies); TAFRO syndrome is mainly reported in Japanese patients. To our knowledge, this is the first pediatric case report detailing a CD-associated disorder progressing to cirrhosis.

CASE SUMMARY

A 10-year old male patient presented with fever and anemia. Six months before hospitalization, he had remarkable abdominal distention. Subsequently, he visited a clinic for a fever that lasted 5 d. The physical findings were marked hepatosplenomegaly and cervical lymphadenopathy. A blood test revealed leukocytosis, microcytic anemia, aspartate aminotransferase-dominant transaminase elevation, high levels of C-reactive protein, polyclonal hypergammaglobulinemia, and high levels of interleukin-6 and vascular endothelial growth factor. Abdominal contrast computed tomography and magnetic resonance imaging suggested cirrhosis, which was confirmed by liver histology. Histological findings in the enlarged hepatic lymph nodes revealed both hyperplasia and atrophy of lymphoid follicles with some vascular hyperplasia and moderate plasmacytosis between the lymphoid follicles, which is compatible with lymph node histology in TAFRO syndrome. Prednisolone was not effective in reducing the patient’s symptoms; therefore, the patient was prescribed tocilizumab. To date, the patient remains free of fever and continues to receive tocilizumab.

CONCLUSION

We described the clinicopathological features of TAFRO syndrome to highlight the clinical presentation of this rare disease in a pediatric case.

Keywords: Castleman disease; Case report; Multi-centric Castleman’s disease; Liver cirrhosis; Tocilizumab; Child

Core tip: Castleman disease (CD) is a lymphoproliferative disorder of unknown cause. TAFRO syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia, anasarca (generalized edema and pleural effusion), fever (pyrexia), reticulin fibrosis (reticulin fibrosis in the bone marrow and the proliferation of megakaryocytes), and organomegaly (e.g., hepatosplenomegaly and multiple lymphadenopathies). To our knowledge, this is the first pediatric case report detailing a CD-associated disorder progressing to cirrhosis.