title: A useful relationship between the presence of extramedullary erythropoeisis and the level of the soluble form of the transferrin receptor in a large cohort of adult patients with thalassemia intermedia: a prospective study.
abstract: In thalassemia intermedia (TI), the increase in bone marrow hemopoietic activity frequently leads to extramedullary erythropoeisis (EMH), but its relationship with the soluble form of transferrin receptor (sTfR) which fully reflects the marrow erythropoietic activity, has not yet been explored. From January 2007 to December 2010, all TI patients attending at our center were prospectively enrolled to undergo sTfR assay and MRI or CT (if claustrophobic) scan evaluation for the presence of paraspinal EMH. A total of 59 patients with TI were studied; EMH involved 23 (39%) patients; overall, the concentration of sTfR varied from 2.6 to 20.6 (mean = 8.7) mg/L, but in splenectomized group and in unsplenectomized group, it varied from 4.2 to 17.8 (mean ± SD = 9.86 ± 3.33) mg/L and from 2.6 to 20.6 (mean ± SD = 7.25 ± 3.9) mg/L, respectively with a statistically significant intergroup difference (p < 0.01). The cutoff point at 8.6 mg/L using the ROC curve showed a sensitivity of 78.3% and a specificity of 72.2%, in predicting EMH but, in unsplenectomized subgroup, they raised to 100% and 90.9%, respectively. These data showed that in TI the level of sTfR could represent a predictive factor of EMH particularly in patients with spleen.
authors.affiliation: UOSD Centro delle Microcitemie "A. Mastrobuoni", AORN A. Cardarelli, Naples, Italy. pabloricchi@libero.it
authors.forename: Paolo
authors.initials: P
authors.lastname: Ricchi
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Ammirabile
authors.forename: Silvia
authors.initials: S
authors.lastname: Costantini
authors.forename: Tiziana
authors.initials: T
authors.lastname: Di Matola
authors.forename: Roberto
authors.initials: R
authors.lastname: Verna
authors.forename: Alvaro
authors.initials: A
authors.lastname: Diano
authors.forename: Maria Carmela
authors.initials: MC
authors.lastname: Foglia
authors.forename: Anna
authors.initials: A
authors.lastname: Spasiano
authors.forename: Patrizia
authors.initials: P
authors.lastname: Cinque
authors.forename: Luciano
authors.initials: L
authors.lastname: Prossomariti
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D004920:Erythropoiesis / Q000502:physiology*; D005260:Female; D006411:Hematopoiesis, Extramedullary / Q000502:physiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D011446:Prospective Studies; D020033:Protein Isoforms / Q000097:blood; D011990:Receptors, Transferrin / Q000032:analysis / Q000097:blood* / Q000737:chemistry; D018401:Sample Size; D012995:Solubility; D055815:Young Adult; D017086:beta-Thalassemia / Q000097:blood* / Q000175:diagnosis* / Q000503:physiopathology*
chemical_list: D020033:Protein Isoforms; D011990:Receptors, Transferrin