Familial Mediterranean Fever After Cord Blood Transplantation for Familial Hemophagocytic Lymphohistiocytosis. Issue 8 (November 2021)

• Record Type: Journal Article
• Authors: Igarashi, Keita; Hori, Tsukasa; Yamamoto, Masaki; Hatakeyama, Naoki; Iesato, Kotoe; Takebayashi, Akira; Kizawa, Toshitaka; Miyamae, Takako; Kawamoto, Manabu; Kawasaki, Yukihiko
• Is Part Of: Journal of pediatric hematology/oncology. Volume 43:Issue 8(2021)
• Publication Date: 2021-11
• Subjects: familial Mediterranean fever -- familial hemophagocytic lymphohistiocytosis -- cord blood transplantation -- colchicine; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000002081
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder accompanied by periodic fever and sterile serositis. We report a 5-year-old boy with FMF, who underwent second unrelated cord blood transplantation (CBT) for recurrent familial hemophagocytic lymphohistiocytosis. Periodic attacks of fever and abdominal pain started 6 months after CBT. He was diagnosed with FMF according to the Tel-Hashomer criteria and treated successfully with colchicine. Genetic testing showed heterozygous p.E148Q mutation in the MEFV gene from both donor and recipient cells. Several CBT-related factors including use of an immunosuppressant can potentially be involved in the pathogenesis of FMF in our patient.