Familial Mediterranean Fever After Cord Blood Transplantation for Familial Hemophagocytic Lymphohistiocytosis.
J Pediatr Hematol Oncol
; 43(8): e1136-e1139, 2021 11 01.
Article
em En
| MEDLINE
| ID: mdl-33560083
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Febre Familiar do Mediterrâneo
/
Transplante de Células-Tronco de Sangue do Cordão Umbilical
/
Linfo-Histiocitose Hemofagocítica
/
Pirina
/
Mutação
Tipo de estudo:
Etiology_studies
/
Prognostic_studies
Limite:
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
J Pediatr Hematol Oncol
Assunto da revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Ano de publicação:
2021
Tipo de documento:
Article