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Tanzeela Farah
Sumaira Khalil
Tariq Ghafoor
Muhammad Tahir
Awais Arshad


OBJECTIVE: To determine the frequency of proptosis and associations of proptosis with demographic, clinical and hematological characteristics with acute myeloid leukemia (AML) in children.

METHODS: This descriptive study was conducted at Combined Military Hospital, Rawalpindi, Pakistan from January 2018 to July 2020. Patients of AML between 1-12 years of age who presented with and without orbital granulocytic sarcomas (OGS), selected by non-probability convenience sampling technique were evaluated for proptosis. Patients were classified into different subtypes of AML according to French-American-British (FAB) classification. Cytogenetic studies by karyotyping were done to identify different genetic abnormalities associated with AML. Kaplan- Meier survival analysis was used to analyze the overall survival of AML patients.

RESULTS: Out of 230 patients diagnosed with AML, 34 (14.78%) patients presented with proptosis. Mean age of presentation was 6.80±3.69 years, with male-female ratio of 2.1:1. Proptosis was unilateral in 19 (55%) and bilateral in 15 (45%) patients. Ten (29.40 %) patients presented primarily with proptosis while 24 (71%) patients presented with proptosis and systemic features of leukemia. Overall AML-M2 was found in 102 (44.3%) cases and other types were observed in 128 (55.6%) cases. Most common FAB AML subtype associated with proptosis was AML-M2 (n=26; 76%). Median duration of survival in AML patients with OGS was 867 days and as compared to 353 days in AML patients without OGS. 

CONCLUSION: Proptosis is a frequent finding in children with AML. AML-M2 is associated with proptosis in children with AML. Survival in patients with OGS was better than patients without OGS.

Article Details

How to Cite
Farah, T., S. Khalil, T. Ghafoor, M. Tahir, and A. Arshad. “PROPTOSIS IN ACUTE MYELOID LEUKEMIA: AN UNDER RECOGNIZED PRESENTATION OF HEMATOLOGICAL MALIGNANCY”. KHYBER MEDICAL UNIVERSITY JOURNAL, Vol. 14, no. 4, Dec. 2021, pp. 211-5, doi:10.35845/kmuj.2021.21005.
Original Articles


1. Metayer C, Dahl G, Wiemels J, Miller M. Childhood leukemia: a preventable disease. Pediatrics 2016;138 (Suppl 1):S45-S55.
2. Hossain MJ, Xie L, Caywood EH. Prognostic factors of childhood and adolescent acute myeloid leukemia (AML) survival: evidence from four decades of US population data. Cancer Epidemiol 2015;39(5):720-6.
3. Taga T, Tomizawa D, Takahashi H, Adachi S. Acute myeloid leukemia in children: Current status and future directions. Pediatr Int 2016;58(2):71-80.
4. Linabery AM, Ross JA. Trends in childhood cancer incidence in the U.S (1992-2004). Cancer 2008;112(2):416-32.
5. Avni B, Koren-Michowitz M. Myeloid sarcoma: current approach and therapeutic options. Ther Adv Hematol 201;2(5):309-16.
6. Noh BW, Park SW, Chun JE, Kim JH, Kim HJ, Lim MK. Granulocytic Sarcoma in the Head and Neck: CT and MR Imaging Findings. Clin Exp Otorhinolaryngol 2009;2(2):66-71.
7. Yilmaz AF, Saydam G, Sahin F, Baran Y. Granulocytic sarcoma: a systematic review. Am J Blood Res 2013;3(4):265-70.
8. Abdallah AM, Abdellatif MA, Elhwary AM. Paediatric orbital tumours in Upper Egypt: A 3-year retrospective analysis at a university hospital. J Clin Ophthalmol 2019; 3 (1): 105-118.
9. Aggarwal E, Mulay K, Honavar SG. Orbital extra-medullary granulocytic sarcoma: clinicopathologic correlation with immunohistochemical features. Surv Ophthalmol 2014;59(2):232-5.
10. Murthy R, Vemuganti GK, Honavar SG, Naik M, Reddy V. Extramedullary leukemia in children presenting with proptosis. J Hematol Oncol 2009;2:4.
11. Stockl FA, Dolmetsch AM, Saornil MA, Font RL, Burnier MN Jr. Orbital granulocytic sarcoma. Br J Ophthalmol 1997;81(12):1084-8.
12. Cavdar AO, Arcasoy A, Babacan E, Gözdaşoğlu S, Topuz U, Fraumeni JF Jr. Ocular granulocytic sarcoma (chloroma) with acute myelomonocytic leukemia in Turkish children. Cancer 1978;41(4):1606-9.<1606::AID-CNCR2820410451>3.0.CO;2-Y.
13. Panda A, Sudan R, Nainiwal S. Childhood proptosis. The invaluable but overlooked peripheral blood smear. Indian J Ophthalmol 2002;50(3):247.
14. Young CW, Ho CS, Chiu NC, Liu HC, Liang DC. Acute myeloid leukemia with initial presentation of facial palsy and exophthalmos. Acta Neurol Taiwan 2016;25(1):18-20.
15. Zimmerman LE, Font RL. Ophthalmologic manifestations of granulocytic sarcoma (myeloid sarcoma or chloroma). The third Pan American Association of Ophthalmology and American Journal of Ophthalmology Lecture. Am J Ophthalmol 1975; 80(6):975-90.
16. Bidar M, Wilson MW, Laquis SJ, Wilson TD, Fleming JC, Wesley RE, et al. Clinical and imaging characteristics of orbital leukemic tumors. Ophthalmic Plast Reconstr Surg 2007;23(2):87-93.
17. Gözdaşoğlu S. Remarks on myeloid sarcoma in children. Turk J Haematol 2019;36(2):122-123.
18. Schwyzer R, Sherman GG, Cohn RJ, Poole JE, Willem P. Granulocytic sarcoma in children with acute myeloblastic leukemia and t(8;21). Med Pediatr Oncol 1998;31(3):144-9.<144::aid-mpo3>;2-b.
19. Felice MS, Zubizarreta PA, Alfaro EM, Gallego MS, Cygler AM, Rosso AD, et al. Good outcome of children with acute myeloid leukemia and t(8;21)(q22;q22), even when associated with granulocytic sarcoma: a report from a single institution in Argentina. Cancer 2000;88(8):1939-44.
20. Ohanian M, Faderl S, Ravandi F, Pemmaraju N, Garcia-Manero G, Cortes J, et al. Is acute myeloid leukemia a liquid tumor? Int J Cancer 2013;133(3):534-43.
21. Johnston DL, Alonzo TA, Gerbing RB, Lange BJ, Woods WG. Superior outcome of pediatric acute myeloid leukemia patients with orbital and CNS myeloid sarcoma: a report from the Children's Oncology Group. Pediatr Blood Cancer 2012;58(4):519-24.
22. Bisschop MM, Révész T, Bierings M, van Weerden JF, van Wering ER, Hählen K, et al. Extramedullary infiltrates at diagnosis have no prognostic significance in children with acute myeloid leukaemia. Leukemia 2001;15(1):46-9.
23. Gözdaşoğlu S, Yavuz G, Unal E, Taçyldz N, Cavdar AO. Orbital granulocytic sarcoma and AML with poor prognosis in Turkish children. Leukemia 2002;16(5):962-3.
24. Kobayashi R, Tawa A, Hanada R, Horibe K, Tsuchida M, Tsukimoto I. Japanese childhood AML cooperative study group. Extramedullary infiltration at diagnosis and prognosis in children with acute myelogenous leukemia. Pediatr Blood Cancer 2007;48(4):393-8.
25. Cuthbertson DW, Punia JN, Owczarzak VL. Myeloid sarcomas of the head and neck in pediatric patients with myeloid leukemia. Ear Nose Throat J 2016;95(9):405-7.