Bone quality in beta-thalassemia intermedia: relationships with bone quantity and endocrine and hematologic variables.
We report the first evaluation of bone quality in 70 thalassemia intermedia (TI) patients (37 males, 33 females, age 41 ± 12 years). Thirty-three patients (47%) had been transfused, 34 (49%) had been splenectomized, 39 (56%) were on iron chelation therapy, and 11 (16%) were on hydroxyurea. Mean hemoglobin was 9.2 ± 1.5 g/dl, median ferritin 537 ng/dl (range 14-4893), and mean liver iron concentration 7.6 ± 6.4 mg Fe/g dw. Fifteen patients (21%) had endocrinopathies, and 29 (41%) had vitamin D deficiency. Bone quantity (bone mineral density, BMD) and bone quality (trabecular bone score, TBS) were evaluated by densitometry. In 53/70 patients (76%), osteopathy was found (osteoporosis in 26/53, osteopenia in 27/53). BMD values were higher in the never-transfused patients and in the not-chelated group. A highly significant correlation was found between splenectomy and BMD at all the sites, with lower values in the splenectomized patients. TBS values were significantly lower in TI patients than in 65 non-thalassemic controls (1.22 vs 1.36, p < 0.01), mainly in those splenectomized and in the transfused and chelated groups (p < 0.01). TBS did not correlate with liver iron concentration values. Our data disclose the major role of non-invasive bone quality evaluation in TI patients, especially those with the worst health state, to obtain a comprehensive assessment of fracture risk. Splenectomy seems to play a major part in bone complications.
Rare Diseases Center, Department of Medicine and Medical Specialties, Ca' Granda Foundation IRCCS Ospedale Maggiore Policlinico, Milan, Italy. marina.baldini@policlinico.mi.it.|UO Medicina Interna, Padiglione Granelli, IRCCS Fondazione Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza 35, 20122, Milan, Italy. marina.baldini@policlinico.mi.it.
Marina
M
Baldini
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
A
A
Marcon
Nuclear Medicine, Bone Metabolic Unit, Ca' Granda Foundation IRCCS Ospedale Maggiore Policlinico, Milan, Italy.
F M
FM
Ulivieri
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
S
S
Seghezzi
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
R
R
Cassin
Scuola di Specializzazione in Radiodiagnostica, University of Milan, Milan, Italy.
C
C
Messina
Rare Diseases Center, Department of Medicine and Medical Specialties, Ca' Granda Foundation IRCCS Ospedale Maggiore Policlinico, Milan, Italy.|Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
M D
MD
Cappellini
Rare Diseases Center, Department of Medicine and Medical Specialties, Ca' Granda Foundation IRCCS Ospedale Maggiore Policlinico, Milan, Italy.
G
G
Graziadei
D015502:Absorptiometry, Photon; D000328:Adult; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000150:complications / Q000378:metabolism; D001842:Bone and Bones / Q000378:metabolism*; D004700:Endocrine System Diseases / Q000150:complications / Q000378:metabolism; D005260:Female; D006801:Humans; D019190:Iron Overload / Q000150:complications / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D010024:Osteoporosis / Q000150:complications / Q000378:metabolism; D017086:beta-Thalassemia / Q000150:complications / Q000378:metabolism*
Osteoporosis; Thalassemia; Trabecular bone score