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Indian Journal of Hematology and Blood Transfusion

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01-09-2017 | Correspondence

Transfusion Dependent Beta-Thalassemia Developing Avascular Necrosis of Femoral Head: A Rare Case

Authors: S. K. Behera, R. K. Jena, S. Sethy, R. K. Nayak, Ashutosh Panigrahi

Published in: Indian Journal of Hematology and Blood Transfusion | Issue 3/2017

Excerpt

Skeletal abnormalities like avascular necrosis (AVN) of hip and bone infarcts are very rarely reported in thalassemia major compared to that in sickle cell anemia. Avascular necrosis (AVN) of femoral head occurs at the incidence of 2.5 per 100 patients yearly in sickle cell anemia (HbSS) [1] which results from recurrent episodes of vaso-occlusion and others factors like endothelial activation, hypercoagulable state, high level of inflammatory mediators, reduced availability of nitrous oxide [2]. …

Milner PF et al (1991) sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med 325:1476–1478CrossRefPubMed

Hacuker H et al (1982) Hematological factors associated with avascular necrosis of the femoral head in homozygous sickle cell disease. Br J Haematol 50:29–34CrossRef

Ingue A, Freeman MA, Vernon-Roberts B, Mizuno S (1976) The pathogenesis of Perthes disease. J Bone Joint Surg 58:453CrossRef

Pinca A, Soriani S, Bezzi TM, Lucci M, Borgatti L (1983) Forme di rarapatologiaosteo-articolarenella thalassemia major. Clin Pediatr 65:1

Vullo C(1982) Talassemiamaior. In: TratattoItaliano di Medicina Interna. 2 edizione, parte 3, vol 3. USES Ed. Scient 1, Firenze, p 2753

Katz K, Horev G, Goshen J, Tamary H (1994) The pattern of bone disease in transfusion dependent thalassemia major patients. Isr J Med Sci 30(8):577–580PubMed

Orzincolo C, Castaldi G, Scutellari PN, Bariani L, Pinca A (1986) Aseptic necrosis of femoral head complicating thalassemia. Skeletal Radiol 15(7):541–544CrossRefPubMed

Title: Transfusion Dependent Beta-Thalassemia Developing Avascular Necrosis of Femoral Head: A Rare Case
Authors: S. K. Behera
R. K. Jena
S. Sethy
R. K. Nayak
Ashutosh Panigrahi
Publication date: 01-09-2017
Publisher: Springer India
Published in: Indian Journal of Hematology and Blood Transfusion / Issue 3/2017
Print ISSN: 0971-4502
Electronic ISSN: 0974-0449
DOI: https://doi.org/10.1007/s12288-016-0747-y

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