• acute kidney injury
• hematology laboratory
• immature platelet fraction
• tafro syndrome
• thrombocytopenia

• TAFRO syndrome
• adrenal lesion
• complete resolution
• cyclosporin
• liver damage
• tocilizumab

• Adult
• Castleman Disease
• Edema
• Humans
• Hyperbilirubinemia
• Liver
• Male
• Panniculitis

• Castleman disease
• TAFRO syndrome
• alkaline phosphatase
• cholangitis

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.

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.

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

• Castleman disease
• Case report
• Multi-centric Castleman’s disease
• Liver cirrhosis
• Tocilizumab
• Child

TAFRO syndrome is a novel disease concept characterized by Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly, multiple lymphadenopathy and a histopathological pattern of atypical Castleman’s disease. A 58-year-old man was diagnosed as TAFRO syndrome by clinical and histopathological findings. After receiving intensive immunosuppressive therapy, his thrombocytopenia and anasarca had not improved. He developed complications such as methicillin-resistant Staphylococcus aureus sepsis, gastrointestinal bleeding, peritonitis caused by Stenotrophomonas maltophilia, gastrointestinal perforation, and disseminated candidiasis resulting in death. Autopsy revealed disseminated candidiasis and hemophagocytic lymphohistiocytosis, with no evidence of TAFRO syndrome. During treatment, we regarded his lasting thrombocytopenia and anasarca as insufficient control of TAFRO syndrome. However, the autopsy revealed that thrombocytopenia was caused by secondary hemophagocytic lymphohistiocytosis caused by over-immunosuppression. We reviewed the published literature to identify indicators of adequate treatment, which suggested improvement of platelet count and anasarca several weeks after initial therapy. This indicated that we could not depend on the platelet count and anasarca in acute medical care after initial treatment. We should treat TAFRO syndrome based on patients’ clinical status and obviate the risk of treatment-related complications caused by over-immunosuppression.

• TAFRO syndrome
• course of treatment
• disseminated candidiasis
• hemophagocytic lymphohistiocytosis
• immunosuppression

• TAFRO syndrome
• ascites
• corticosteroid
• hemodialysis
• rituximab
• thrombocytopenia

• Ascites/drug therapy
• Drug Therapy, Combination
• Edema/diagnosis
• Edema/drug therapy
• Female
• Fever/diagnosis
• Glucocorticoids/therapeutic use
• Humans
• Immunologic Factors/therapeutic use
• Lymphadenopathy/diagnosis
• Lymphadenopathy/drug therapy
• Middle Aged
• Primary Myelofibrosis/diagnosis
• Primary Myelofibrosis/drug therapy
• Remission Induction
• Renal Dialysis
• Renal Insufficiency/diagnosis
• Renal Insufficiency/therapy
• Rituximab/therapeutic use
• Syndrome
• Thrombocytopenia/diagnosis
• Thrombocytopenia/drug therapy
• Withholding Treatment