Abstract

Myeloid sarcoma is a tumor of myeloblast or poorly differentiated myeloid cells that can occur in extramedullary sites, as isolated tumor, concomitantly with or after the diagnosis of acute myeloblastic leukemia. Despite considerable improvement achieved in the outcome of patients with acute myeloblastic leukemia by using intensive chemotherapy and/or hematopoietic stem cell transplantation, it is generally considered to be poor in patients with myeloid sarcoma. The aim of this review is to indicate the potential therapeutic effects of high-dose glucocorticoid treatment which induce differentiation and apoptosis of myeloid leukemic cells, as a new treatment approach for patients with myeloid sarcoma. We have shown that short-course (3 to7 days) high-dose methylprednisolone treatment that might also induce differentiation and apoptosis of leukemic cells in the extramedullary sites resulted in dramatic decreases in the size of myeloid sarcoma in children with or without marrow infiltration. From the results of our long-term clinical studies, we suggest that to use short- course high-dose glucocorticoids combined with intensive chemotherapy protocol would be a promising treatment strategy for patients with myeloid sarcoma. However, in further studies, the prognostic significance of different localization of myeloid sarcoma and the long term effect of the addition of highdose glucocorticoid to intensive AML chemotherapy protocols should be explored in larger series.

Highlights

  • Myeloid sarcoma is a tumor of myeloblast or poorly differentiated myeloid cells that can occur in extramedullary sites, as isolated tumor, concomitantly with or after the diagnosis of acute myeloblastic leukemia

  • The role of radiotherapy on survival is not clear, the results of several studies revealed that addition of radiotherapy to chemotherapy or surgical removal of leukemic mass has no effect on the outcome, but, these may be indicated for the relief of symptomatic complications of tumor mass [1,4,5,10,12]

  • We first demonstrated in 1991, morphologic evidence of terminal differentiation of myeloid leukemic cells in a boy with acute myeloblastic leukemia (AML)-M4 who presented with orbitaocular Myeloid sarcoma (MS) treated with high-dose methylprednisolone (HDMP, 20-30 mg/ kg/day, not exceeding 1 g/day) orally in a single dose

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Summary

Introduction

Myeloid sarcoma is a tumor of myeloblast or poorly differentiated myeloid cells that can occur in extramedullary sites, as isolated tumor, concomitantly with or after the diagnosis of acute myeloblastic leukemia. The use of agents that can induce differentiation and apoptosis of myeloid leukemic cells would be a promising treatment approach for patients with MS.

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