Liver cirrhosis in a child associated with Castleman's disease: A case report  

作　　者：Soya Kobayashi Ayano Inui Tomoyuki Tsunoda Syuichiro Umetsu Tsuyoshi Sogo Masaaki Mori Masato Shinkai Tomoo Fujisawa 

机构地区：[1]Department of Pediatric Hepatology and Gastroenterology,Saiseikai Yokohama City Tobu Hospital,Yokohama 230-8765,Japan [2]Department of Lifetime Clinical Immunology,Graduate School of Medical and Dental Sciences,Tokyo Medical and Dental University,Tokyo 113-8519,Japan [3]Department of Surgery,Kanagawa Children’s Medical Center,Yokohama 232-8555,Japan

出　　处：《World Journal of Clinical Cases》2020年第9期1656-1665,共10页世界临床病例杂志

基　　金：Kazuyuki Yoshizaki,Department of Organic Fine Chemicals,The Institute of Scientific and Industrial Research,Osaka University,Osaka,Japan.Masaru Kojima,Department of Diagnostic Pathology,Dokkyo Medical University School of Medicine,Tochigi,Japan.

摘　　要：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.

关 键 词：CASTLEMAN DISEASE Case report Multi-centric Castleman’s DISEASE Liver cirrhosis TOCILIZUMAB CHILD 

分 类 号：R725.7[医药卫生—儿科]