Abstract
The skull in homozygous β-thalassaemia may present several abnormalities, such as osteopenia, widening of the diploic space, and a “hairon-end” appearance. In some cases it presents also a particular stratified appearance caused by a variable number of osseous lamellae, parallel with the inner table. This “lamellated skull” was observed in 16 out of 150 patients affected by the disease (10.6%). Possible mechanisms are discussed. The lamellar osseous changes could be due to repeated periosteal osteoblastic reactions to the sinusoidal neovascularization associated with marrow hyperplasia in poorly transfused patients.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Baker HD (1964) Roentgen manifestations of Cooley's anemia. Ann NY Acad Sci 119:641
Caffey J (1951) Cooley's erythroblastic anemia. Some skeletal findings in adolescents and young adults. AJR 65: 547
Caffey J (1957) Cooley's anemia: A review of the roentgenographic findings in the skeleton. AJR 78:381
lawson JP (1986) Thalassemia. In: Radiology. Lippincott, Philadelphia
Modell B, Berdoukas V (1984) The clinical approach to thalassaemia. Grune and Stratton, London
Moseley JE (1963) Bone changes in hematologic disorders (roentgen aspects). Grune and Stratton, New York
Piomelli S, Karpatkin MH, Arzanian M, Zamani M, Becker MH, Geneiser N, Danoff SJ, Kuhns WJ (1974) Hypertransfusion regimen in patients with Cooley's anemia. Ann NY Acad Sci 232:186
Putschar WG (1983) The skeletal pathology of thalassemia. In: Radiology. Lippincott, Philadelphia
Reimann F, Kuran S (1973) Ursache Entstehung und Wesen des, “Burstensymptoms” am Schädel bei schweren Erkrankungen des Blutes. Virchows Arch [A] 358:173
Resnick D, Niwayama G (1988) Diagnosis of bone and joint disorders. Saunders, Philadelphia
Silverman FN (1987) Caffey's pediatric X-ray diagnosis: An integrated imaging approach. Yearbook Medical Publisher, Chicago
Warwich RX, Williams PL (1973) Gray's anatomy, 35th British edn. Saunders, Philadelphia
Wolman IJ (1964) Transfusion therapy in Cooley's anemia: growth and health as related to long-range hemoglobin levels. A progress report. Ann NY Acad Sci 119:736
Wolman IJ, Ortolani M (1969) Some clinical features of Cooley's anemia patients as related to transfusion schedules. Ann NY Acad 165:407
Zaino EC (1980) Pathophysiology of thalassaemia. Ann NY Acad Sci 344:284
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Orzincolo, C., Castaldi, G., Scutellari, P.N. et al. The “lamellated” skull in β-thalassaemia. Skeletal Radiol 18, 373–376 (1989). https://doi.org/10.1007/BF00361428
Issue Date:
DOI: https://doi.org/10.1007/BF00361428