Pediatric Castleman Disease Combined with TAFRO Syndrome: Two Case Reports and Literature Review

Abstract

AbstractObjective To investigate the clinical characteristics, treatment, and prognosis for TAFRO syndrome in pediatric patients. Method A retrospective analysis was performed on two patients with TAFRO Syndrome at Beijing Children’s Hospital, Beijing, China. One case was reported in 2019 as Castleman disease (CD) combined with hemophagocytic lymphohistiocytosis (HLH)[1]. The case was examined again from the perspective of TAFRO syndrome, and the follow-up was updated. Clinical features, treatment, and prognosis data of these patients were collected. Also, the published literature on pediatric TAFRO syndrome was reviewed. Results Two male patients were enrolled in this study. The ages of onset were 2 years and 9 years, respectively. Both patients presented fever, anemia, thrombocytopenia, lymphadenopathy, hepatosplenomegaly, ascites, bilateral pleural effusion, and limb edema and were diagnosed with idiopathic multicentric CD (iMCD) combined with TAFRO syndrome and secondary HLH. One patient responded satisfactorily to BCD (Bortezomib, Cyclophosphamide, and Dexamethasone) chemotherapy and thalidomide therapy, while the other had an adequate response to tocilizumab; their conditions were controlled by the last follow-up. Conclusion Due to the complicated clinical manifestations of TAFRO syndrome and the severe consequences, its diagnosis and treatment are challenging in clinical practice. Multiple chemotherapies combined with immunomodulatory or targeted drugs affect the prognosis of TAFRO syndrome. Also, the co-existing HLH should be noted in the course of the disease.