Blood Res 2017; 52(3): 184-192  https://doi.org/10.5045/br.2017.52.3.184
Clinical characteristics and treatment outcomes of isolated myeloid sarcoma without bone marrow involvement: a single-institution experience
Jung Yeon Lee1, Haerim Chung2, Hyunsoo Cho3, Ji Eun Jang2, Yundeok Kim2, Soo-Jeong Kim2, Jin Seok Kim2, Shin Young Hyun1, Yoo Hong Min2, June-Won Cheong2
Department of Internal Medicine, 1Yonsei University Wonju College of Medicine, Wonju, 2Yonsei University College of Medicine, Seoul, 3Graduate School of Medical Science and Engineering, Korea Advanced Institute of Science and Technology, Daejeon, Korea
Correspondence to: June-Won Cheong, M.D., Ph.D. Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul 03722, KoreaE-mail: jwcheong70@yuhs.ac
Received: April 12, 2017; Revised: May 20, 2017; Accepted: June 17, 2017; Published online: September 30, 2017.
© The Korean Journal of Hematology. All rights reserved.

Abstract
Background 
Isolated myeloid sarcoma (MS) is a rare extramedullary tumor mass composed of malignant myeloid precursor cells without any evidence of leukemia in the peripheral blood and bone marrow. We describe the clinical characteristics and outcomes of patients diagnosed with isolated MS at our institution.
Methods 
We retrospectively reviewed 9 of 497 acute myeloid leukemia (AML) patients (1.8%) with isolated MS. Isolated MS patients were divided into 2 groups according to the first-line treatment strategy: systemic treatment only (S) or local treatment with or without systemic treatment (LS).
Results
The most common site of MS occurrence was the head and neck area (N=4, 44.4%), followed by the anterior mediastinum (N=2, 22.2%) and the gastrointestinal tract (N=2, 22.2%). The tumors of 4 patients (44.4%) eventually evolved to AML, in a median time of 13.4 months (range, 2.4‒20.1 mo). The number of patients achieving complete remission after first-line treatment was higher in the LS group (N=5, 83.3%) than in the S group (N=1, 33.3%) (P =0.226). All patients in the LS group survived, but those in the S group died (P=0.012).
Conclusion
Accurate and rapid diagnosis using various modalities and the early initiation of intensive combined treatment may be the optimal strategies to reduce the risk of isolated MS subsequently evolving to AML. To fully understand the characteristics of isolated MS, a larger number of patients from a multinational study is necessary.
Keywords: Acute myeloid leukemia, Myeloid sarcoma, Treatment


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