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Chapter 31

The Hemoglobinopathies:

Sickle Cell Disease and Thalassemia


Abramson H, et al: Sickle Cell Disease. Diagnosis, Management, Education and Research. St. Louis, CV Mosby Co, 1973.

Adams A, McKie V, Nichols F, et al: The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 326:605-610, 1992.

Adams RJ, Aaslid R, Gammal TE, et al: Detection of cerebral vasculopathy in sickle cell disease using transcranial doppler ultrasonography and magnetic resonance imaging: case report. Stroke 19:518-520, 1988.

Adedeji MO, Okafor LA, Ejele OA, Monu JUV: Sickle cell cephalohaematoma in Benin City, Nigeria - a report of three cases. Cent Afr J Med 34:33-37, 1988.

Aksoy M, et al: Roentgenographic bone changes in chronic iron deficiency anemia: a study of 12 patients. Blood 27:677-686, 1966.

Allison AC: Protection afforded by the sickle-cell trait against subtertian malarial infection. Br Med J 1:290-292, 1954.

Amundsen TR, Siegel MJ, Siegel BA: Osteomyelitis and infarction in sickle cell hemoglobinopathies: differentiation by combined technetium and gallium scintigraphy. Radiology 153:807-812, 1984.

Baker DH: Roentgen manifestations of Cooley's anemia. Ann NY Acad Sci 119:641-661, 1964.

Barton CJ, Cockshott WP: Bone changes in Hb SC disease. Am J Roentgenol 88:523-532, 1962.

Bannerman RM, et al: Thalassemia intermedia with iron overload, cardiac failure, diabetes mellitus, hypopituitarism and porphyrinuria. Am J Med 42: 476-486,1967.

Beltran J, Herman LJ, Burk JM, et al: Femoral head avascular necrosis: MR imaging with clinical-pathologic and radio-nuclide correlation. Radiology 166:215-220, 1988.

Bohrer SP: Acute long-bone diaphyseal infarcts in sickle cell disease. Br J Radiol 43:685-697, 1970.

Bohrer SP: Bone infarcts in sickle cell anaemia. Tropenmed Parasitol 16:299-312, 1971.

Bohrer SP: Fractures complicating bone infarcts and/or osteomyelitis in sickle cell disease. Clin Radiol 23:83-88, 1971.

Bohrer SP: Bone Ischaemia and Infarction in Sickle Cell Disease. St. Louis, Warren H. Green, Inc, 1981.

Bohrer SP: Bone changes in the extremities in sickle cell anemia. Semin Roentgenol 22:176-185, 1987.

Borosh L, et al: Large cerebral vessel disease in sickle cell anemia. Radiology 125:850, 1977.

Bowman HS, Procopio F: Hereditary non-spherocytic hemolytic anemia of the pyruvate-kinase deficient type. Ann Intern Med 58:567-591, 1963.

Brill PW, Winchester P, Giardina PJ, Cunningham-Rundles S: Desferrioxamine-induced bone dysplasia in patients with thalassemia major. AJR 156:561-565, 1991.

Brody AS, Sorette MP, Gooding CA, et al: Induced alignment of flowing sickle erythrocytes in a magnetic field: a preliminary report. Invest Radiol 20:560-566, 1985.

Brody AS, Embury SH, Mentzer WC, et al: Preservation of sickle cell blood-flow patterns during MR imaging: an in vivo study. AJR 151:139-141, 1988.

Caffey J: Cooley's erythroblastic anemia: some skeletal findings in adolescents and young adults. Am J Roentgenol 65:547-560, 1951.

Caffey J: Cooley's anemia: a review of the roentgenographic findings in the skeleton. Am J Roentgenol 78:381-391, 1957.

Castleman B, McNeely B: Case records of the Massachusetts General Hospital. N Engl J Med 278:782-791, 1968.

Chatterjea JB: Some aspects of haemoglobin E and its genetic interaction with thalassemia. Indian J Med Res 53:1-23, 1965.

Cheung MC, Goldberg JD, Kan YW: Prenatal diagnosis of sickle cell anaemia and thalassaemia by analysis of fetal cells in maternal blood. Nature Genetics 14:264-268, 1996.

Close AS, et al: Spinal cord compression due to extramedullary hematopoiesis. Ann Intern Med 48:421, 1958.

Cockshott WP, Weaver EJM: Primary tropical splenic abscess: a misnomer. Brit J Surg 49:665-669, 1962.

Cockshott WP: Dactylitis and growth disorders. Brit J Radiol 36:19-26, 1963.

Cockshott WP: Splenic abscesses, in Middlemiss H (ed): Tropical Radiology. London, William Heinemann Medical Books Ltd, 1961, pp 205-208.

Cockshott WP, Middlemiss H: Clinical Radiology in the Tropics. Edinburgh, Churchill Livingstone, 1979, pp 73-81.

Coleman BG, Kressel HY, Dalinka MK, et al: Radiology 168:525-528, 1988.

Currarino G, Erlandson ME: Premature fusion of epiphyses in Cooley's anemia. Radiology 83:656-664, 1964.

Da Costa JL, et al: Extramedullary haemopoiesis with multiple tumour-simulating mediastinal masses in hemoglobin E-thalassaemia disease. Chest 65:210, 1974.

Davey WW: Sickle cell anemia, in Companion to Surgery in Africa. Edinburgh, E & S Livingstone Ltd, 1968, pp 1-6.

Dihlmann W: CT analysis of the upper end of the femur: the asterisk sign and ischemic bone necrosis of the femoral head. Skeletal Radiol 8:251-258, 1982.

Edeiken J, Hodes PJ: Roentgen Diagnosis of Diseases of Bone. 2nd edn. Baltimore, Williams & Wilkins Co, 1973.

Edington GM, et al: The abnormal hemoglobins and the abnormal hemoglobin diseases, in Pathology in the Tropics. Baltimore, Williams & Wilkins Co, 1969, pp 362-410.

Engle MA: Cardiac involvement in Cooley's anemia. Ann N Y Acad Sci 119:694-702, 1964.

Ennis JT, Serjeant GR, Middlemiss JH: Homozygous sickle cell disease in Jamaica. Brit J Radiol 46:943-950.

Feldman F, Zwass A, Staron RB, Haramati N: MRI of soft tissue abnormalities: a primary cause of sickle cell crisis. Skeletal Radiol 22:501-506, 1993.

Fishbone G, Nunez D, Leon R, et al: Massive splenic infarction in sickle cell hemoglobin C disease-angiographic findings. Am J Roentgenol 129:927-928, 1976.

Fleischer RA, Rubler S: Primary cardiomyopathy in non anaemic patients. Association with sickle cell trait. Am J Cardiol 22:532-537, 1968.

Gatto I, et al: Compression sul midollo spinale da proliferazione di midollo osteanello spazio epidurale in soggetto affectto da mallattia de Cooley splenectomizzato. Haematologica 38:61,1954.

Genez BM, Wilson MR, Houk RW, et al: Early osteonecrosis of the femoral head: detection in high-risk patients with MR imaging. Radiology 168:521-524, 1988.

Gillespy T III, Genant HK, Helms CA: Magnetic resonance imaging of osteonecrosis. Radiol Clin North Am 24:193-208, 1986.

Glickstein MF, Burk DL Jr, Schiebler ML, et al: Avascular necrosis versus other diseases of the hip: sensitivity of MR imaging. Radiology 169:213-215, 1988.

Golding JSR, et al: Bone changes in sickle cell anemia and its genetic variants. J Bone Joint Surg 41-B:711-718, 1959.

Gückel F, Brix G, Semmler W, et al: Proton chemical shift imaging of bone marrow for monitoring therapy in leukemia. J Comput Assist Tomogr 14:954-959, 1990.

Hanam E: Massive thoracic extramedullary haematopoiesis in a case of haemoglobin E-thalassemia. Singapore Med J 5:122, 1964.

Hardjasudarma M, Black-Payne C, Wood M, Ganley J: Orbital bone infarcts in sickle cell anemia. Proceedings of 19th International Congress of Radiology, Beijing, 1996, p 80.

Hatrick AG, Howlett DC, Jarosz JM, Irvine AT: Neurological complications in sickle cell disease: a pictorial review. Poster 0201. Brit Institute Radiol Annual Conf, 1997. Radiology 1997, Suppl to Vol 70, Brit J Radiol, 1997.

Hendrickse RG, Collard P: Salmonella osteitis in Nigerian children. Lancet 1:80-82, 1960.

Hill AV, Bowden DK, O'Shaughnessy DF, et al: Beta thalassemia in Melanesia: association with malaria and characterization of a common variant. Blood 72:9-14, 1988.

Hook EW, et al: Salmonella osteomyelitis in patients with sickle cell anemia. N Engl J Med 257:403-407, 1957.

Huehns ER, Shooter EM: Human haemoglobins. J Med Genet 2:48-90, 1965.

Hurrow BR, Sloane IA, Liebman: Roentgenologic demonstration of renal papillary necrosis in sickle cell trait. NEJM 268:969-976, 1963.

Ibrahim SA: Sickle cell thalassemia disease in Sudanese. East Afr Med J 47:47-49, 1970.

Jacobson AF, Harley JD, Lipsky BA, Pecoraro RE: Diagnosis of osteomyeltis in the presence of soft-tissue infection and radiologic evidence of osseous abnormalities: value of leukocyte scintigraphy. AJR 157:807-812, 1991.

Jones SR, Binder RA, Donowho EM: Sudden death in sickle cell trait. NEJM 282:323-325, 1970.

Kagwa-Nyanzi A: Causes of death among sickle cell anaemia patients at Mulago Hospital, Kampala (Uganda). East Afr Med J 47:337-343, 1970.

Kangarloo Iooshang, Dietrich RB, Taira RT, Gold RH, et al: MR imaging of bone marrow in children. J Comput Assist Tomogr 10:205-209, 1986.

Kaplan KR, Mitchell DG, Steiner RM, et al: Polycythemia vera and myelofibrosis: correlation of MR imaging, clinical, and laboratory findings. Radiology 183:329-334, 1992.

Kim HC, Alavi A, Russell MO, Schwartz E: Differentiation of bone and bone marrow infarcts from osteomyelitis in sickle cell disorders. Clin Nucl Med 14:249-254, 1989.

Knoblich R: Extramedullary hematopoiesis presenting as intrathoracic tumors. Report of a case with thalassemia minor. Cancer 13:462-468, 1960.

Kogutt MS, Goldwag SS, Gupta KL, et al: Correlation of transcranial Doppler ultrasonography with MRI and MRA in the evaluation of sickle cell disease patients with prior stroke. Pediatric Radiology 24:204-206, 1994.

Kolawole TM, Bohrer SP: Sickle cell trait and leg ulceration. Br Med J 2:603, 1970.

Korsten J, Grossman H, Winchester PH, Canale VC: Extramedullary haematopoiesis in patients with thalassemia anemia. Radiology 95:257-263, 1970.

Kricun ME: Red-yellow marrow conversion: its effect on the location of some solitary bone lesions. Skeletal Radiol 14:10-19, 1985.

Lagundoye SB: Radiological features of sickle cell anaemia and related hemoglobinopathies in Nigeria. Afr J Med Sci 1:315-342, 1970.

Lagundoye SB: Splenic calcification in sickle cell disease in Nigerians. Trop Geogr Med 23:135-140, 1971.

Laird L, Dezateux C, Anionwu EN: Neonatal screening for sickle cell disorders: what about carrier infants? Brit Med J 313:407-411, 1996.

Lambotte C: Sickle cell anemia, in Jelliffe DB: Diseases of Children in the Subtropics and Tropics-2nd edn. London, Edward Arnold Publishers Ltd, 1970, pp 531-540.

Lang P, Jergesen HE, Moseley ME, et al: Avascular necrosis of the femoral head: high-field-strength MR imaging with histologic correlation. Radiology 169:517-524, 1988.

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

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

Lawson JP, Ablow RC, Pearson HA: Premature fusion of the proximal humeral epiphyses in thalassemia. AJR 140:239-244, 1983.

Lawson JP: Thalassemia. In: Taveras JM, Ferrucci JT (eds). Radiology. Philadelphia, JB Lippincott, 1986, pp 1-7.

Lee A, Thomas P, Cupidore L, et al: Improved survival in homozygous sickle cell disease: lessons from a cohort study. Brit Med J 311:1600-1602, 1995.

Loiacono PJ, Reeder MM: Case of the month from the AFIP: Thalassemia major (Cooley's anemia). Radiology 92:385-394, 1969.

Luhby AL, Cooperman JM: Folic acid deficiency in thalassemia major. Lancet 2:490-491, 1961.

Macht SH, Roman PW: The radiologic changes in sickle cell anaemia. Radiology 51:697-707, 1948.

Mankad VP, Yang Y-M, Williams JP, Brogdon BG: Magnetic resonance imaging of bone marrow in sickle cell patients. Am J Pediatr Hematol Oncol 10:344-347, 1988.

Marwick C: Trial halted as sickle cell treatment proves itself. JAMA 273:611, 1995.

McCall IW, Moule N, Desai P, Serjeant GR: Urographic findings in homozygous sickle cell disease. Radiology 126:99-104, 1978.

Michael D, Mason DO, Zlatkin MB, et al: Radiology 173:355-359, 1989.

Middlemiss JH: Sickle cell anemia. J Fac Radiol Lond 9:16-24, 1958. Middlemiss JH, Raper AB: Skeletal changes in the haemoglobinopathies. J Bone Joint Surg 48B:693-702, 1966.

Mihindukulasuriya JCL, et al: A case of paraparesis due to extramedullary haemopoiesis in HbE-thalassemia. Postgrad Med J 53:397-399, 1977.

Mitchell DG, Rao VM, Dalinka MK, et al: Femoral head avascular necrosis: correlation of MR imaging, radiographic staging, radionuclide imaging, and clinical findings. Radiology 162:709-715, 1987.

Mitchell DG, Kundel HL, Steinberg ME, et al: Avascular necrosis of the hip: comparison of MR, CT, and scintigraphy. AJR 147:67-71, 1986.

Mitchell R, Fupi F: Sickling in Tanzania. East Afr Med J 49:638-642, 1972.

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Munk PL, Helms CA, Holt RG: Immature bone infarcts: findings on plain radiographs and MR scans. AJR 152:547-549, 1989.

Ndugwa CM, Mukidi F: Sickle-cell cephalohaematoma. East Afr Med J 53:545-548, 1976.

Ng LM, Liebman J, Ansiovar J, Gross S: Cardiovascular findings in children with sickle cell anaemia. Dis Chest 52:788-799, 1967.

O'Hara AE: Roentgenographic osseous manifestations of the anaemias and the leukemias. Clin Orthoped 52:63-82, 1967.

Olufemi-Williams A, Lagundoye SB, Johnson CL: Lamellations of the diploe in the skulls of patients with sickle cell anaemia. Arch Dis Childhood 50:948-952, 1975.

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Serjeant GR, Ennis JT, Middlemiss JH: Haemoglobin SC disease in Jamaica. Brit J Radiol 46:935-942, 1973.

Serjeant GR, Ennis JT, Middlemiss JH: Sickle cell B thalassaemia in Jamaica. Brit J Radiol 46:951-959, 1973.

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