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A Reappraisal of Some Skeletal Changes in Currently Treated β-Thalassemia

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Book cover Radiology of Thalassemia

Abstract

It is common knowledge that in β-thalassemic syndromes bone alterations are absent at birth, but may be appreciated radiologicallly after the 1st year of life and become characteristic during early childhood [3, 4,10,11, 41, 52, 55, 73, 82]. Bone marrow hyperplasia is generally considered responsible for skeletal changes [12, 14, 18, 27, 30, 50, 77] (osteopenia with widening of the medulla, thinning of the cortex in long bones and reactive periosteal new bone formation). These alterations, described by Cooley and Lee in 1925, are diagnostic and characteristic in the severe forms of anemia (homozygous β-thalassemia). They involve the entire skeleton but are usually more conspicuous in the skull and extremities [4, 10, 11, 41, 52, 53, 55]. With advancing age, skeletal lesions disappear in the peripheral segments, where red marrow is converted into yellow marrow, but they persist in the axial skeleton, where the bone marrow remains red. Therefore, in adolescence and early adulthood bone changes are particularly common in the skull, pelvis, and spine. Pseudotumors of extramedullary hematopoiesis may occur in paravertebral sites and in many other organs and tissues [8, 22, 45, 57, 58]. This process is far more frequent in thalassemia intermedia [13, 66].

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References

  1. Andresen J, Nielsen HE (1986) Amount of bone and bone remodelling in normal subjects and in patients with chronic renal failure. Acta Radiol (Diagn) (Stockh) 27: 341–346

    CAS  Google Scholar 

  2. Asbell MB (1964) Orthodontic aspects of Coo ley’s anemia. Ann NY Acad Sci 119: 662–663

    Article  PubMed  CAS  Google Scholar 

  3. Astaldi G, Tolentino P, Sacchetti C (1951) La talassemia, Tipografia del Libro, Pavia

    Google Scholar 

  4. Baker DH (1964) Roentgen manifestations of Cooley’s anemia. Ann NY Acad Sci 119: 641–661

    Article  PubMed  CAS  Google Scholar 

  5. Barnett E, Nordin BEC (1960) The radiological diagnosis of osteoporosis: a new approach. Clin Radiol 11: 166–174

    Article  PubMed  CAS  Google Scholar 

  6. Barry M, Flynn DM, Letsky EA, Risdon RA (1974) Long-term chelation therapy in thalassemia major: effect on liver iron concentration, iron histology and clinical progress. Br Med J 2: 16–25

    Article  PubMed  CAS  Google Scholar 

  7. Beard MEJ, Necheles TF, Allen DM (1969) Clinical experience with intensive transfusion therapy in Cooley’s anemia. Ann NY Acad Sci 165: 415–422

    Article  PubMed  CAS  Google Scholar 

  8. Bron LJ, Paquelet JR, Tetalman MR (1980) Intrathoracic extramedullary hematopoiesis: appearance on 99m ‘re sulfur colloid marrow scan. AJR 134: 1254–12255

    Google Scholar 

  9. Brook CGD, Thompson EN, Marshall WC, Whitehouse RH (1969) Growth in children with thalassemia major and effect of two different transfusion regimens. Arch Dis Child 44: 612–615

    Article  PubMed  CAS  Google Scholar 

  10. Caffey J (1951) Cooley’s erythroblastic anemia. Some skeletal findings in adolescent and young adults. AJR 65: 547–560

    CAS  Google Scholar 

  11. Caffey J (1957) Cooley’s anemia: a review of the roentgenographic findings in the skeleton. AJR 78: 381–391

    CAS  Google Scholar 

  12. Cajozzo A, Perricone R, Tolomeo M (1985) Le talassemie. Haematologica (Pavia) 70: 341–358

    Google Scholar 

  13. Camascella C, Saglio G, Pich PG, Canavoso P, Ferraris R, Bianco G, Mazza U (1981) Thoracic extramedullary hematopoiesis in thalassemia intermedia during adult life: a report of 9 cases. Haematologica (Pavia) 66: 613–626

    Google Scholar 

  14. Cammisa M, Sabella G (1967) Considerazioni clinico-radilogiche sulla patogenesi delle alterazioni ossee nella talassemia major. Nunt Radiol 33: 77–101

    Google Scholar 

  15. Canale VC, Steinherz P, New M, Erlandson M (1974) Endocrine function in thalassemia major. Ann NY Acad Sci 232: 333–345

    Article  PubMed  CAS  Google Scholar 

  16. Cao A, Rosatelli MC (1985) Diagnosi prenatale di emoglobinopatie ereditarie. Haematologica (Pavia) 70: 361–368

    Google Scholar 

  17. Caronia F, Mancuso L, Rigano P, Castellano S, Bondi F, Maggio A (1985) Danno d’organo da accumulo de ferro in pazienti talassemici. Haematologica (Pavia) 70: 383–390

    Google Scholar 

  18. Castaldi G (1986) Talassemie, In: Baserga A (ed) Ematologica clinica. UTET, Torino, pp 111–132

    Google Scholar 

  19. Costin G, Kogut MD, Hyman CB, Ortega JA (1979) Endocrine abnormalities in thalassemia major. Am J Dis Child 133: 497–502

    PubMed  CAS  Google Scholar 

  20. Citarella P, Marco P, Pecorella M, Cajozzo A (1979) La splenectomia nella talassemia grave ed intermedia. Atti X XVI Congr Naz Soc Ital Ernatol, Torino

    Google Scholar 

  21. Currarino G, Erlandson ME (1964) Premature fusion of epiphysis in Cooley’s anemia. Radiology 83: 656–664

    PubMed  CAS  Google Scholar 

  22. Danza FM, Falappa P, Leone G, Pincelli G (1982) Extramedullary hematopoiesis. AJR 139: 837–838

    PubMed  CAS  Google Scholar 

  23. Dequeker J (1980) Measurement of bone mass and bone remodelling in vivo. Value of the radiogrammetric approach. Acta Rheumatol 4: 40–75

    Google Scholar 

  24. Diness DM, Canale VC, Arnold WD (1976) Fractures in thalassemia. J Bone Joint Surg (Am) 58: 662–666

    Google Scholar 

  25. Doxiadis S, Georgaki E, Papamichael D, Papadakou-Lagogianni S, Lapatsanis P (1978) Bone density in thalassemic children during the course of the disease. Pediat Res 12: 811–815

    Article  PubMed  CAS  Google Scholar 

  26. Evans RA, McDonnel GD, Schieb M (1978) Metacarpal cortical area as an index of bone mass. J. Radiol 51: 428–431

    Article  CAS  Google Scholar 

  27. Fessas P, Loukopoulos D (1974) Theß-thalassemias. Clin Haematol 3: 411–440

    Google Scholar 

  28. Fink H (1964) Transfusion hemochromatosis in Cooley’s anemia. Ann NY Acad Sci 119: 680–685

    Article  PubMed  CAS  Google Scholar 

  29. Garn SM (1981) Contributions of the radiographic image to our knowledge of human growth. AJR 137: 231–239

    PubMed  CAS  Google Scholar 

  30. Gabutti V, Piga A, Cordero A, Fortina P. Times F (1981) Erythropoiesis in thalassemia. Haematologica (Pavia) 66: 257–268

    Google Scholar 

  31. Fink IJ, Pastakia B, Barranger JA (1984) Enlarged phalangeal nutrient foramina in Gaucher disease andß-thalassemia major. AJR 143: 647–949

    PubMed  CAS  Google Scholar 

  32. Flink DM, Fairney A, Jackson D, Clayton BE (1976) Hormonal changes in thalassemia major. Arch Dis Child 51: 828–836

    Article  Google Scholar 

  33. Gratwick GM, Bullough PG, Bohne WH, Markenson AL, Peterson CM (1978) Thalassemic osteoarthropathy. Ann Intern Med 88: 494–501

    PubMed  CAS  Google Scholar 

  34. Johnston FE, Krogman WM (1964) Patterns of growth in children with thalassemia major. Ann NY Acad Sci 119: 667–679

    Article  PubMed  CAS  Google Scholar 

  35. Johnston FE, Rosemann JM (1967) The effects of more frequent transfusions upon bone loss in thalassemia major. Pediat Res 1: 479–483

    Article  PubMed  CAS  Google Scholar 

  36. Kaplan RI, Werther R, Castano FA (1964) Dental and oral findings in Cooley’s anemia: a study of fifty cases. Ann NY Acad Sci 119: 664–666

    Article  PubMed  CAS  Google Scholar 

  37. Lassman MN, O’Brien RT, Pearson HA, Wise JK, Donabedian RK, Felig P, Genel A (1974) Endocrine evaluation in thalassemia major. Ann NY Acad Sci 232: 226–237

    Article  PubMed  CAS  Google Scholar 

  38. Lawson JP, Ablow RC, Pearson HA (1981) The ribs in thalassemia. I. The relationship to therapy, Radiology 140: 663–672

    CAS  Google Scholar 

  39. Lawson JP, Ablow RC, Pearson HA (1981) The ribs in thalassemia. II. The pathogenesis of the changes. Radiology 140: 673–679

    CAS  Google Scholar 

  40. Lawson JP, Albow RC, Pearson HA (1984) Calvarial and phalangeal vascular impressions in thalassemia. AJR 143: 641–645

    PubMed  CAS  Google Scholar 

  41. Lawson JP (1986) Thalassemia, in radiology, diagnosis, imaging, intervension, Taveras and Ferrucci, vol. 5. Lippincott and Harper Philadelphia, pp 1–7

    Google Scholar 

  42. Leccisotti A, Meloni T (1967) Sui rapporti fra le alteraziono ossee e la maturazione scheletrica nell anemia mediterranea. Nunt Radiol 33: 179–202

    Google Scholar 

  43. Logothetis J, Loewenson RB, Augoustaki O, Economidou J, Costantoulakis M (1972) Body growth in Cooley’s anemia (homozygous betathalassemia) with correlative study as to other aspects of the illness in 138 cases. Pediatrics 50: 92–99

    PubMed  CAS  Google Scholar 

  44. Logothetis J, Economidou J, Costantoulakis M, Augoustaki O, Loewenson RB, Bilek M (1971) Cephalofacial derformities in thalassemia major ( Cooley’s anemia). AM J Dis Child 121: 300–306

    PubMed  CAS  Google Scholar 

  45. Long JA, Doppman JL, Nienhuis AW (1980) Computed tomographic studies of thoracic extramedullary hematopoieses, J Comput Assa Tom 4: 67–70

    Article  Google Scholar 

  46. Lucarelli G, Polchi P, Galimberti M, Delfini C, Giardini C, Angelucci E, Politi P, Baronciani D, Manenti F, La Nasa G (1985) Trapianto di midollo osseo nella thalassemia, Haematologica (Pavia) 70: 407–413

    Google Scholar 

  47. Masera G (1985) La terapia delle talassemie. Regime transfusionale: aspetti clinici. Haematologica (Pavia) 70: 377–381

    Google Scholar 

  48. Miniero R, Sacchetti L, Isolato V(1977) L’accresimento staturo-ponderale nella ß-talassemia omozigote in rapporto al regime transfusionale. Min Ped 29: 73–79

    CAS  Google Scholar 

  49. Model B, Berdoukas V (1984) The clinical approach to thalassemia. Grune and Stratton, London

    Google Scholar 

  50. Montuori R, de Ritis V, Lupi L, di Girolamo R, Quattrin N (1977) Lo scheletro nella microcitemia e nella microdrepanocitosi. Rilievi dello studio di 133 casi. Atti XX VII Congr Naz Soc Ital Ematol, Torino

    Google Scholar 

  51. Morgan DB (1973) The metacarpal bone: a comparison of the various indices for the assessment of the amount of bone and for the detection of loss of bone. Clin Radiol 24: 77–82

    Article  PubMed  CAS  Google Scholar 

  52. Moseley JE (1963) Bone changes in hematologic disorders, Grune and Stratton, New York

    Google Scholar 

  53. Moseley JE (1974) Skeletal changes in the anemias. Semin Roentgenol 9: 169–195

    Article  PubMed  CAS  Google Scholar 

  54. Orsini A, Perrimond H, Pierron HH, Charvet JP, Sebag F, Rebuffel P (1973) Risultati a lungo termine della terapia del morbo di Cooley. Prospect Pediatr 11: 415–421

    Google Scholar 

  55. Ortolani M, Cremonese M (1963) Le alterazioni scheletriche nel morbo die Cooley. Clin Ortop 15: 599–614

    Google Scholar 

  56. Orzincolo C, Castaldi G, Scutellari PN, Bariani L, Pinca A (1986) Aseptic necrosis of femoral head complicating thalassemia. Skeletal Radiol 15: 541–544

    Article  PubMed  CAS  Google Scholar 

  57. Papavasiliou CG (1965) Tumor simulating intrathoracic extramedullary hemopoiesis. Clinical and roentgenologic considerations. AJR 93: 695–702

    CAS  Google Scholar 

  58. Papavasiliou C, Gouliamos A, Andreou J (1986) The marrow heterotopia in thalassemia. Eur J Radiol 6: 92–96

    PubMed  CAS  Google Scholar 

  59. Papavasiliou C, Konstantoulakis M, Priovolos J, Labrakos E, Agrogiannis B, Manettas E, Papaodysseas S, Pippis P (1979) The anterior costal margin mass in patients with thalassemia major. Clin Radiol 30: 521–524

    Article  PubMed  CAS  Google Scholar 

  60. Piomelli S, Danoff SJ, Becker MH, Lipera MJ, Travis SF (1969) Prevention of bone malformations and cardiomegaly in Cooley’s anemia by early hypertransfusion regimen, Ann NY Acad Sci 165: 427–436

    Article  PubMed  CAS  Google Scholar 

  61. 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: 186–192

    Article  PubMed  CAS  Google Scholar 

  62. Resnick D (1981) Hemoglobinopathies and other anemias. In: Resnick D, Niwayama G (eds.) Diagnosis of bone and joint disorders. Saunders, Philadelphia

    Google Scholar 

  63. Rodolico G, Cavallaro V, Minutolo V, Di Cataldo A (1985) La splenectomia nel trattamento della talassemia. Haematologica (Pavia) 70: 399–405

    Google Scholar 

  64. Russo G (1985) Terapia ferrochelante della talassemia. Haematologica (Pavia) 70: 391–398

    Google Scholar 

  65. Schon JB, Radel E (1964) Transfusion therapy and its complications inpatients with Cooley’s anemia. Ann NY Acad Sci 119: 703–708

    Google Scholar 

  66. Scutellari PN, Bighi S, Franceschini F, Orzincolo C (1982) Le alterazioni scheletriche delle betatalassemie intermedie, Studio clinico e radiologico. Minn Med 73: 457–466

    CAS  Google Scholar 

  67. Scutellari PN, Bighi S, Orzincolo C, Nibbio A (1981) Stato attuale delle alterazioni scheletriche delle beta-talassemie. Riv Emoter ImmunoematoI 28: 39–66

    Google Scholar 

  68. Scutellari PN, Franceschini F, Orzincolo C (1981) Skeletal changes in treated ß-thalassemias: a reappraisal. Rays 7: 13–25

    Google Scholar 

  69. Scutellari PN, Orzincolo C, Bighi S (1981) Studio xeroradiografico delle alterazioni ossee nelle beta-talassemie. Radiol Med (Torino) 67: 913–919

    CAS  Google Scholar 

  70. Scutellari PN, Orzincolo C, Calzolari F (1983) Aspetti radiologici delle alterazioni odonto-stomatologiche nellß-talassemie. Minerva Stomatol 32: 433–440

    PubMed  CAS  Google Scholar 

  71. Scutellari PN, Orzincolo C, Calzolari F, Tilotta F (1983) Le coste nelle ß-talassemie. Evoluzione delle alterazioni in rapporto alla terapia transfusionale. Radiol Med (Torino) 69: 654–660

    CAS  Google Scholar 

  72. Scutellari PN, Orzincolo C, Tamarozzi R (1985) Xeradiography inß-thalassemia. Skeletal Radiol 13: 39–43

    Article  PubMed  CAS  Google Scholar 

  73. Silvestroni E, Bianco I, Brancati C (1971) Le emoglobinopatie mediterranee. Recenti Progr Med 51: 212–249

    PubMed  CAS  Google Scholar 

  74. Tas I, Smith P, Cohen T (1976) Metric and morphologic characteristic of the dentition in beta thalassaemia major in man Arch Oral Biol 21: 583–586

    CAS  Google Scholar 

  75. Toti A (1951) Le fratture traumatiche del morbo die Cooley. Ann Radiol (Bologna) 23: 81–92

    CAS  Google Scholar 

  76. Vullo C (1981) Treatment and prognosis of thalassemias, Haematologica 66: 293–308

    Google Scholar 

  77. Weatherall DJ, Clegg JB (1981) the thalassaemia syndromes.Blackwell Scientific Oxford

    Google Scholar 

  78. Weatherall DJ, Clegg JB (1982) Thalassaemia revisited. Cell 29: 7–9

    Article  PubMed  CAS  Google Scholar 

  79. Wolff JA, Luke KH (1969) Management of thalassaemia. A comparative programm. Ann NY Acad Sci 165: 423–426

    Article  PubMed  CAS  Google Scholar 

  80. 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: 736–747

    Article  PubMed  CAS  Google Scholar 

  81. Solmann IJ, Ortoloani M (1969) Some clinical features of Cooley’s anemia patients as related to transfusion schedules. Ann NY Acad Sci 165: 407–414

    Article  Google Scholar 

  82. Zaino EC (1980) Pathophysiology of thalassemia. Ann NY Acad Sci 344: 284–303

    Article  PubMed  CAS  Google Scholar 

  83. Zaino EC, Kuo B, Roginsky MS (1969) Growth retardation in thalassemia major. Ann NY Acad Sci 165: 394–399

    Article  PubMed  CAS  Google Scholar 

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  1. P. N. Scutellari
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  2. F. Franceschini
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  3. C. Orzincolo
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Editors and Affiliations

  1. Department of Radiology, University of Athens, School of Medicine, Areteion Hospital, 115 28, Athens, Greece

    Constantin Papavasiliou  & Theophanis Cambouris  & 

  2. Department of Internal Medicine, University of Athens, School of Medicine, 115 28, Athens, Greece

    Phaedon Fessas

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Scutellari, P.N., Franceschini, F., Orzincolo, C. (1989). A Reappraisal of Some Skeletal Changes in Currently Treated β-Thalassemia. In: Papavasiliou, C., Cambouris, T., Fessas, P. (eds) Radiology of Thalassemia. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-72587-6_6

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  • DOI: https://doi.org/10.1007/978-3-642-72587-6_6

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