GEOFFREY M. GRATWICK, M.D.; PETER G. BULLOUGH, M.D.; WALTHER H. O. BOHNE, M.D.; ALICEJANE L. MARKENSON, M.D.; CHARLES M. PETERSON, M.D., F.A.C.P.
Patients with beta thalassemia major may develop a specific osteoarthropathy as they approach the second and third decades of life. Twenty-five of 50 patients between the ages of 5 and 33 years had evidence of periarticular disease that consisted of dull-aching ankle pain exacerbated by weight bearing and relieved by rest. Involvement was symmetrical with mild swelling and pain on bone compression. Arthrocentesis showed no evidence of inflammation. Radiographic changes included osteopenia, widened medullary spaces, thin cortices with coarse trabeculations, and evidence of microfractures. Histologic studies confirmed the presence of microfractures and showed osteomalacia and an increase in osteoblastic and osteoclastic surface area with iron deposits at the calcification front and cement lines. The relative roles of iron overload, divalent cation metabolism, erythroid hyperplasia, or other factors in the pathogenesis of the syndrome remain unknown, and no specific therapy can be recommended at present.
Learn more about subscription options.
Register Now for a free account.
GRATWICK GM, BULLOUGH PG, BOHNE WHO, MARKENSON AL, PETERSON CM. Thalassemic Osteoarthropathy. Ann Intern Med. 1978;88:494–501. doi: 10.7326/0003-4819-88-4-494
Download citation file:
Published: Ann Intern Med. 1978;88(4):494-501.
Hematology/Oncology, Red Cell Disorders.
Copyright © 2017 American College of Physicians. All Rights Reserved.
Print ISSN: 0003-4819 | Online ISSN: 1539-3704
Conditions of Use
This PDF is available to Subscribers Only