Abstract
We performed a retrospective study describing the characteristics of myeloid sarcoma (MS) and evaluated the outcome of hematopoietic stem cell transplantation (HSCT) in patients with MS. There were 27 patients with de novo isolated MS, 34 with de novo leukemic MS and 13 with secondary leukemic MS in our study. Sixty-three patients received induction chemotherapy. Following induction therapy, 35 patients underwent HSCT, including 10 autogenous HSCT (auto-HSCT) and 25 allogeneic HSCT (allo-HSCT) cases. Compared with intensive chemotherapy only as consolidation treatment, HSCT (auto-/allo-HSCT) significantly improved the overall survival (OS) of MS patients (p < 0.05), while allo-HSCT also improved progression-free survival (PFS, p = 0.032). According to multivariate analysis, poorer prognosis in terms of OS was observed in older patients (p = 0.024, HR = 1.030, 95% CI 1.004–1.057), while HSCT (auto/allo-HSCT) had a favorable impact on OS for patients with MS (auto-HSCT, p = 0.044, HR = 0.201, 95% CI 0.042–0.959; allo-HSCT, p = 0.038, HR = 0.341, 95% CI 0.124–0.943). Extramedullary disease without complete remission (CR) after induction therapy was the sole variable independent of high OS and PFS (p = 0.049, HR = 2.243, 95% CI: 1.005–5.005; p = 0.017, HR = 2.535, 95% CI 1.180–5.448, respectively). The data indicate that HSCT is an effective treatment for patients with MS who have achieved CR of extramedullary disease after induction therapy.
Similar content being viewed by others
References
Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391–405.
Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary acute myeloid leukemia. Blood. 2011;118:3785–93.
Byrd JC, Weiss RB, Arthur DC, Lawrence D, Baer MR, Davey F, et al. Extramedullary leukemia adversely affects hematologic complete remission rate and overall survival in patients with t(8;21)(q22;q22): results from Cancer and Leukemia Group B 8461. J Clin Oncol. 1997;15:466–75.
Döhner H, Estey EH, Amadori S, Appelbaum FR, Büchner T, Burnett AK, et al. Diagnosis and management of acute myeloid leukemia in adults: recommendations from an international expert panel, on behalf of the European LeukemiaNet. Blood. 2010;115:453–74.
Huter O, Brezinka C, Nachbaur D, Schwaighofer H, Lang A, Niederwieser D. Successful treatment of primary extramedullary leukemia (EML) of the uterus with radical surgery, chemotherapy, autologous bone marrow transplantation (BMT) and prophylactic local irradiation. Bone Marrow Transplant. 1996;18:663–4.
Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia. 2007;21:340–50.
Chevallier P, Mohty M, Lioure B, Michel G, Contentin N, Deconinck E, et al. Allogeneic hematopoietic stem-cell transplantation for myeloid sarcoma: a retrospective study from the SFGM-TC. J Clin Oncol. 2008;26:4940–3.
Chevallier P, Labopin M, Cornelissen J, Socié G, Rocha V, Mohty M, et al. Allogeneic hematopoietic stem cell transplantation for isolated and leukemic myeloid sarcoma in adults: a report from the Acute Leukemia Working Party of the European group for Blood and Marrow Transplantation. Haematologica. 2011;96:1391–4.
Shimizu H, Saitoh T, Tanaka M, Mori T, Sakura T, Kawai N, et al. Allogeneic hematopoietic stem cell transplantation for adult AML patients with granulocytic sarcoma. Leukemia. 2012;26:2469–73.
Lazzarotto D, Candoni A, Filì C, Forghieri F, Pagano L, Busca A, et al. (2017) Clinical outcome of myeloid sarcoma in adult patients and effect of allogeneic stem cell transplantation. Results from a multicenter survey. Leuk Res 53: 74–81.
Bourlon C, Lipton JH, Deotare U, Gupta V, Kim DD, Kuruvilla J, et al. Extramedullary disease at diagnosis of AML does not influence outcome of patients undergoing allogeneic hematopoietic cell transplant in CR1. Eur J Haematol. 2017;99:234–9.
Döhner H, Estey E, Grimwade D, Amadori S, Appelbaum FR, Büchner T, et al. Diagnosis and management of AML in adults: 2017 ELN recommendations from an international expert panel. Blood. 2017;129:424–47.
Tsimberidou AM, Kantarjian HM, Estey E, Cortes JE, Verstovsek S, Faderl S, et al. Outcome in patients with nonleukemic granulocytic sarcoma treated with chemotherapy with or without radiotherapy. Leukemia. 2003;17:1100–3.
Gordon MY, Blackett NM. Reconstruction of the hematopoietic system after stem cell transplantation. Cell Transplant. 1998;7:339–44.
Schoemans HM, Lee SJ, Ferrara JL, Wolff D, Levine JE, Schultz KR, et al. EBMT-NIH-CIBMTR Task Force position statement on standardized terminology & guidance for graft-versus-host disease assessment. Bone Marrow Transplant. 2018;53:1401–15.
Simon R, Makuch RW. A non-parametric graphical representation of the relationship between survival and the occurrence of an event: application to responder versus non-responder bias. Stat Med. 1984;3:35–44.
Mantel N, Byar DP. Evaluation of response-time data involving transient States: an illustration using heart-transplant data. Public Am Stat Assoc. 1974;69:81–6.
Tsimberidou AM, Kantarjian HM, Wen S, Keating MJ, O’Brien S, Brandt M, et al. Myeloid sarcoma is associated with superior event-free survival and overall survival compared with acute myeloid leukemia. Cancer. 2008;113:1370–8.
Yamauchi K, Yasuda M. Comparison in treatments of nonleukemic granulocytic sarcoma: report of two cases and a review of 72 cases in the literature. Cancer. 2002;94:1739–46.
Shimizu H, Saitoh T, Hatsumi N, Takada S, Yokohama A, Handa H, et al. Clinical significance of granulocytic sarcoma in adult patients with acute myeloid leukemia. Cancer Sci. 2012;103:1513–7.
Xu LH, Wang Y, Chen ZY, Fang JP. Myeloid sarcoma is associated with poor clinical outcome in pediatric patients with acute myeloid leukemia. J Cancer Res Clin Oncol. 2020;146:1011–20.
Vodyanik MA, Thomson JA, Slukvin II. Leukosialin (CD43) defines hematopoietic progenitors in human embryonic stem cell differentiation cultures. Blood. 2006;108:2095–105.
Broyde A, Boycov O, Strenov Y, Okon E, Shpilberg O, Bairey O. Role and prognostic significance of the Ki-67 index in non-Hodgkin’s lymphoma. Am J Hematol. 2009;84:338–43.
Tallman MS, Hakimian D, Shaw JM, Lissner GS, Russell EJ, Variakojis D. Granulocytic sarcoma is associated with the 8;21 translocation in acute myeloid leukemia. J Clin Oncol. 1993;11:690–7.
Psiachou-Leonard E, Paterakis G, Stefanaki K, Mikraki-Christou V, Haidas S. Cerebellar granulocytic sarcoma in an infant with CD56+ acute monoblastic leukemia. Leuk Res. 2001;25:1019–21.
Falini B, Lenze D, Hasserjian R, Coupland S, Jaehne D, Soupir C, et al. Cytoplasmic mutated nucleophosmin (NPM) defines the molecular status of a significant fraction of myeloid sarcomas. Leukemia. 2007;21:1566–70.
Ansari-Lari MA, Yang CF, Tinawi-Aljundi R, Cooper L, Long P, Allan RH, et al. FLT3 mutations in myeloid sarcoma. Br J Haematol. 2004;126:785–91.
Willekens C, Renneville A, Broutin S, Saada V, Micol JB, Delahousse J, et al. Mutational profiling of isolated myeloid sarcomas and utility of serum 2HG as biomarker of IDH1/2 mutations. Leukemia. 2018;32(9):2008–81.
Kashofer K, Gornicec M, Lind K, Caraffini V, Schauer S, Beham-Schmid C, et al. Detection of prognostically relevant mutations and translocations in myeloid sarcoma by next generation sequencing. Leuk Lymphoma. 2018;59:501–4.
Lazarevic V, Orsmark-Pietras C, Lilljebjörn H, Pettersson L, Rissler M, Lübking A, et al. Isolated myelosarcoma is characterized by recurrent NFE2 mutations and concurrent preleukemic clones in the bone marrow. Blood. 2018;131:577–81.
Acknowledgements
Dr. Depei Wu and Xiang Zhang contributed to the conception of the study and manuscript revision. Meng Shan, Yutong Lu and Menglu Yang contributed to collecting and performing the data analysis and preparing the manuscript. Peng Wang, Shenqi Lu and Luwei Zhang helped collect and perform data analysis and prepare the manuscript. Huiying Qiu, Suning Chen and Yang Xu contributed to the data analysis and manuscript revision. All the data were from Jiangsu Biobank of Clinical Resources.
Funding
This work was supported by grants from the National Natural Science Foundation of China (81730003, 81870120), the Natural Science Foundation of Jiangsu Province (BK20171205), the Social Development Project of Jiangsu Province (BE2019655), the Priority Academic Program Development of Jiangsu Higher Education Institutions (PAPD), and the National Key Research and Development Program (2017ZX09304021, 2017YFA0104500 and 2019YFC0840604).
Author information
Authors and Affiliations
Corresponding authors
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Shan, M., Lu, Y., Yang, M. et al. Characteristics and transplant outcome of myeloid sarcoma: a single-institute study. Int J Hematol 113, 682–692 (2021). https://doi.org/10.1007/s12185-021-03081-2
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12185-021-03081-2