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Thalassemic Osteoarthropathy

GEOFFREY M. GRATWICK, M.D.; PETER G. BULLOUGH, M.D.; WALTHER H. O. BOHNE, M.D.; ALICEJANE L. MARKENSON, M.D.; and CHARLES M. PETERSON, M.D., F.A.C.P.
[+] Article and Author Information

Grant support: in part by grants from the National Institutes of Health (AM 5-2202, AM 18894, and HL 20016); The Children's Blood Foundation; The Cooley's Anemia Volunteers; The Order of AHEPA and The Lincoln Fund.

▸Requests for reprints should be addressed to Charles M. Peterson, M.D.; The Rockefeller University, 1230 York Ave.; New York, NY 10021.


New York, New York


© 1978 American College of PhysiciansAmerican College of Physicians


Ann Intern Med. 1978;88(4):494-501. doi:10.7326/0003-4819-88-4-494
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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.

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