title: Immunosuppressive therapy for kidney transplant prevents vaso-occlusive crisis in a haemoglobin SC disease patient.
abstract: Although the molecular basis of sickle cell disease (SCD) is well established, the wide variability in clinical manifestations still puzzles haematologists and clinicians. Recently, SCD started to be considered by different groups as a chronic inflammatory condition, where the inflammatory tendency of each individual could drive more or less severe clinical features. Here we describe a haemoglobin SC disease patient (heterozygous to both HbS and HbC variants) that experienced several vaso-occlusive crises before underwent a successful kidney transplantation. Since then (16 years ago), she is on uninterruped immunosuppressive therapy, and do not experienced any severe vaso-occlusive crisis. Considering SCD associated morbidity as a result of exacerbated immune responses, we suggest that the immunosuppressive therapy directed to the kidney graft maintenance is actually also helping in the control of the chronic inflammatory responses associated to SCD.
authors.affiliation: Immunogenetics Laboratory, Department of Genetics, Universidade Federal do Rio Grande do Sul - UFRGS, Av Bento Gonçalves 9500, Predio 43 323, Caixa Postal 15053, CEP 91501-970, Porto Alegre, RS, Brazil. jabchies@terra.com.br
authors.forename: J A B
authors.initials: JA
authors.lastname: Chies
authors.forename: C
authors.initials: C
authors.lastname: Dresch
authors.forename: M S
authors.initials: MS
authors.lastname: Cruz
authors.forename: G
authors.initials: G
authors.lastname: da Silva
authors.forename: E
authors.initials: E
authors.lastname: Barros
authors.forename: C
authors.initials: C
authors.lastname: Bittar
authors.forename: J
authors.initials: J
authors.lastname: Friedrisch
authors.forename: L M R
authors.initials: LM
authors.lastname: Silla
mesh_terms: D000328:Adult; D005260:Female; D006084:Graft Rejection / Q000209:etiology / Q000517:prevention & control*; D006450:Hemoglobin SC Disease / Q000150:complications / Q000188:drug therapy* / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery; D016030:Kidney Transplantation / Q000009:adverse effects; D018448:Models, Immunological; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000209:etiology / Q000276:immunology* / Q000517:prevention & control*
chemical_list: D007166:Immunosuppressive Agents