ORAK HÜCRE ANEMİ KRİZİNE SEKONDER BİLATERAL FEMUR BAŞI OSTEONEKROZUNUN KEMİK SİNTİGRAFİSİ İLE DEĞERLENDİRİLMESİ

Orak hücreli anemi (Sicklecellanemia) hemoglobin S olarak adlandırılan anormal hemoglobinlerin sebeb olduğu herediter geçişli hastalık olarak tanımlanmaktadır. Orak hücre anemi krizinde genellikle göğüste, kemiklerde ve abdomendeki kapillerlerin morfolojisi bozulmuş eritrositler tarafından tıkanmasıyla meydana gelen infarktlar oluşur. Bu vaka takdimindeorak hücre anemisi bulunan ve bir aydır devam eden yürümekte zorluk ve ağrı şikayeti bulunan 29 yaşında erkek hasta sunulmuştur.Orak hücreli anemi (Sicklecellanemia) hemoglobin S olarak adlandırılan anormal hemoglobinlerin sebeb olduğu vasooklisiv, aplastik ve hemolitik krizlere yol açabilen herediter geçişli bir hastalıktır (1). Orak hücreli anemide akut (ağrılı vasooklisiv kriz) ve kronik olarak (avaskuler nekrozun progresiv olarak ilerlemesi) kemik tutulumu en sık karşılaşılan komplikasyonlardan biridir (2).

The Evaluation of Bilateral Femoral Head Osteonecrosis Secondary to Sickle Cell Anemia Crisiswith Bone Scintigraphy

Sicklecellanemia is defined as a hereditary disease which is caused by abnormal hemoglobins that are called hemoglobin S. In sickle cell anemia crisis, generally infarction develops as a result of the blockage of capillaries in the chest, bones and abdomen by the erythrocytes with damaged morphology. In this case presentation, a 29 year old male patient who has sickle cell anemia and who has been experiencing pain and difficulty in walking for a month is chosen. Sicklecellanemia is defined as a hereditary disease which is caused by abnormal hemoglobins that are called hemoglobin S. In sickle cell anemia crisis, generally infarction develops as a result of the blockage of capillaries in the chest, bones and abdomen by the erythrocytes with damaged morphology. In this case presentation, a 29 year old male patient who has sickle cell anemia and who has been experiencing pain and difficulty in walking for a month is chosen. 

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  • 1. Cerci SS, Suslu H, Cerci C, Yildiz M, Ozbek FM, Balci TA, Yesildag A, Canatan D. Different findings in Tc-99m MDP bone scintigraphy of patients with sickle cell disease: report of three cases.AnnNuclMed.2007;21(5):311-4.
  • 2. Almeida A, Roberts I. Bone involvement in sickle cell disease. Br J Haematol. 2005;129(4):482-90.
  • 3. Ganesh, A., William, R.R., Mitra, S., Yanamadala, S., Hussein, S.S., Al Kindi, S., et al. Orbital involvement in sickle cell disease: a report of five cases and review literature. Eye. 2001;15:774–780.
  • 4. Ataga, K.I. ,Orringer, E.P. Bone marrow necrosis in sickle cell disease: a description of three cases and a review of the literature. AmericanJournal of the Medical Sciences. 2000;320:342–347.
  • 5. Demirbas, K.A.,Aktener, B.O. ,Unsal, C. Pulpal necrosis with sickle cell anaemia. International Endodontic Journal.2004;37:602–606.
  • 6. Frenette, P.S. Sickle cell vasoocclusion: heterotypic, multi cellular aggregations driven by leukocyte adhesion. Microcirculation. 2004;11:167–177.
  • 7. Ejindu VC, Hine AL, Mashayekhi M, Shorvon PJ, Misra RR. Musculoskeletal manifestations of sickle cell disease. Radiographics. 2007;27(4):1005-21.
  • 8. Lonergan, G.J.,Cline, D.B. ,Abbondanzo, S.L. Sicklecellanemia. Radiographics.2001; 21: 971–994.
  • 9. Sebes JI. Diagnostic imaging of bone and joint abnormalities associated with sickle cell hemoglobinopathies. AJR Am J Roentgenol. 1989;152(6):1153-59.
  • 10. Mankad, V.N., Williams, J.P., Harpen, M.D., Manci, E., Longenecker, G., Moore, R.B., Shah, A., Yang, Y.M. ,Brogdon, B.G. Magnetic resonance imaging of bone marrow in sicklecelldisease: clinical, hematologic, and pathologic correlations. Blood.1990;75:274– 283.
  • 11. Frush, D.P.,Heyneman, L.E., Ware, R.E. , Bissett, G.S.. MR features of soft-tissue abnormalities due to acute marrow infarction in five children with sickle cell disease. AJR AmericanJournal of Roentgenology.1999;173:989–993.
  • 12. Kim, S.K., Miller, J.H. Natural history and distribution of bone and bone marrow infarction in sickle hemoglobinopathies. Journal of NuclearMedicine. 2002;43:896–900.
  • 13. Milner PF, Kraus AP, Sebes JI, Sleeper LA, Dukes KA, Embury SH, Bellevue R, Koshy M, Moohr JW, Smith J. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325(21):1476-81.
  • 14. Mackenzie JD, Hernandez A, Pena A, Ruppert K, Khrichenko D, Gonzalez L, Jawad AF, Wells L, Smith-Whitley K, Jaramillo D. Magnetic resonance imaging in children with sickle cell disease--detecting alterations in theapparent diffusion coefficient in hips with a vascularnecrosis. Pediatr Radiol. 2012;42(6):706-13