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Extra-medullary hematopoiesis causing bilateral optic atrophy in beta thalassemia/Hb E disease

Ittipunkul, N.; Martin, T.; Siriwanasan, R.; Olanratanachai, K.; Rootman, J.

Journal of the Medical Association of Thailand 90(4): 809-812

2007

ISSN/ISBN: 0125-2208
PMID: 17487139
Document Number: 2854
Objective: To report a rare presentation of bilateral optic nerve compression from extra-medullary hematopoiesis in beta thalassemia/Hb E disease.Design: Interventional case report.Clinical presentation: A 13-year-old Thai girl was reported with slowly progressive bilateral visual loss due to optic neuropathy as a result of compression from extra-medullary hematopoiesis. Computed tomography of orbit and brain revealed a homogeneous enhancing midline soft tissue mass originating from the ethmoid and sphenoid sinuses along with extensive involvement of the skull.Intervention: Blood transfusion once every month and low-dose radiotherapy to sphenoid and ethmoid bones with some visual improvement.Conclusion: Extra-medullary hematopoiesis in beta thalassemia can involve paranasal sinuses and cause compressive neuropathy. In patients with beta thalassemia that required multiple blood transfusions with progressive visual loss, ectopic marrow compression of optic nerve should be ruled out.

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Extra-medullary hematopoiesis causing bilateral optic atrophy in beta thalassemia/Hb E disease

Tantraworasin, A.; Saeteng, S. 2013: Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with beta thalassemia hemoglobin e disease Journal of the Medical Association of Thailand 96(7): 866-869
Junien-Lavillauroy, C.; de Marigny, G.; Bargem; Harachon, R. 1970: Giant ethmoido-sphenoidal mucocele with bilateral optic atrophy Journal Francais d'Oto-Rhino-Laryngologie Audio-Phonologie et Chirurgie Maxillo-Faciale 19(8): 668-669 Passim
Newton, K.L.; McNeeley, S.G.; Novick, M. 1983: Extramedullary hematopoiesis presenting as a pelvic mass in a patient with beta-thalassemia intermedia JAMA 250(16): 2178-2179
Habibzadeh, F.; Yadollahie, M.; Haghshenas, M. 2005: Extra-medullary haematopoiesis in beta-thalassaemia Journal of Postgraduate Medicine 51(1): 45-46
Ben-Bassat, I.; Hertz, M.; Selzer, G.; Ramot, B. 1977: Extramedullary hematopoiesis with multiple tumor-simulating mediastinal masses in a patient with beta-thalassemia intermedia Israel Journal of Medical Sciences 13(12): 1206-1210
Drukker, J.; Lindenburg, P.A. 1972: Bilateral optic atrophy in a one and one-half-year-old child possibly a result of administration of iodochlorhydroxyquinoline (Enterovioform) Nederlands Tijdschrift Voor Geneeskunde 116(36): 1618-1620
Yu, Y.C.; Kao, E.L.; Chou, S.H.; Lin, T.J.; Chien, C.H. 1991: Intrathoracic extramedullary hematopoiesis simulating posterior mediastinal mass--report of a case in patient with beta-thalassemia intermedia Gaoxiong Yi Xue Ke Xue Za Zhi 7(1): 43-48
Issaragrisil, S.; Wanachiwanawin, W.; Bhuripanyo, K.; Benjasuratwong, Y.; Piankijagum, A.; Wasi, P. 1987: Infection in thalassemia: a retrospective study of 1,018 patients with beta-thalassemia/Hb E disease Birth Defects Original Article Series 23(5a): 505-511
Indrák, K.; Divoký, V.; Brabec, V.; Chrobák, L.; Mociková, K.; Sakalová, A.; Svobodová, M.; Indráková, J.; Hammerová, T.; Zarnovicanová, M. 1994: Dominant beta-thalassemia alleles in the Czech and Slovak population (beta-thalassemia mutations in 112(T-A) and 121(G-T) codons and the unstable Hradec Králové hemoglobin or alpha 2 beta 2 115 (G17) Ala-Asp) Vnitrni Lekarstvi 40(4): 223-230
Lebedeva, I.V.; Ivanovskaia, M.G.; Fedorov, A.N.; Limborskaia, S.A.; Shabarova, Z.A. 1994: A new method of nonradioactive labelling of oligonucleotides and their use as allele-specific probes for detecting mutations causing beta-thalassemia Molekuliarnaia Biologiia 28(4): 796-804
Orisaka, M.; Koyamada, K.; Suzuki, Z.; Itoh, T.; Harano, K.; Harano, T. 1998: Beta(+)-thalassemia (-31CapA-->G) homozygosity discovered on the mass medical examination: diagnosis for beta-thalassemia and DNA analysis Rinsho Byori. Japanese Journal of Clinical Pathology 46(3): 259-264
Limborskaia, S.A.; Bukhman, V.L.; Prosniak, M.I.; Fedorov, A.N.; Slominskiĭ, P.A. 1987: Molecular causes of thalassemia. IV. Cloning of the beta-globin gene in a patient with beta-thalassemia from Azerbaijan and determination of the point mutation in a minor intron Genetika 23(2): 228-238
Viprakasit, V.; Tanphaichitr, V.S.; Mahasandana, C.; Assteerawatt, A.; Suwantol, L.; Veerakul, G.; Kankirawatana, S.; Pung-Amritt, P.; Suvatte, V. 2001: Linear growth in homozygous beta-thalassemia and beta-thalassemia/hemoglobin e patients under different treatment regimens Journal of the Medical Association of Thailand 84(7): 929-941
Tatu, T.; Sritong, W.; Sa-Nguansermsri, T. 2014: The associations of SEA-alpha thalassemia 1, XmnI-Ggamma polymorphism and beta-globin gene mutations with the clinical severity of beta-thalassemia syndrome in northern Thailand Journal of the Medical Association of Thailand 97(3): 300-307
Zhao, Y.; Xu, X. 2001: Alpha2(CD31 AGG-->AAG, Arg-->Lys) causing non-deletional alpha-thalassemia in a Chinese family with HbH disease Haematologica 86(5): 541-542
Sebastián de Erice, M.; Romero López, J.; Navarro Esteban, J.; Jiménez, F.; Soto Faure, L. 1981: Hereditary optic atrophies. Study of a family with dominant autosomal optic atrophy Revista Clinica Espanola 163(5): 341-343
Cavallacci, G. 1972: Vascular pathogenesis of the optic atrophy in indirect traumatology of the optic nerve Rivista di Neurologia 42(4): 337-354
Kupch, I.A.; Dagile, I.A. 1985: Bilateral extra-intracranial microanastomoses in moyamoya disease Zhurnal Voprosy Neirokhirurgii Imeni N. N. Burdenko 2: 50-51
Wilson, D.R.; Kline, L.B. 1992: Leber's hereditary optic neuropathy with bilateral distended optic nerve sheaths Journal of Clinical Neuro-Ophthalmology 12(1): 73-74
Garcin, R.; Raverdy, P.; Delthil, S.; Man, H.X.; Chimenes, H. 1961: On a heredo-familial disease combining cataract, optic atrophy, extrapyramidal symptoms and certain defects of Friedreich's disease. (Its nosological position in relation to the Behr's syndrome, the Marinesco-Sjogren syndrome and Friedreich's disease with ocular symptoms Revue Neurologique (Paris) 104: 373-379