Clinical characteristics, treatment, and prognosis of 118 cases of myeloid sarcoma

Abstract

Myeloid sarcoma is a rare manifestation of acute myeloid leukemia (AML) and is associated with poor overall survival (OS). The optimal treatment remains unclear. The study retrospectively evaluated 118 patients with myeloid sarcoma who were treated at the First Affiliated Hospital of Zhengzhou University from January 2010 to July 2021. All cases were diagnosed by tissue biopsy. 41 patients underwent genetic mutation analysis. The most frequent genetic mutations were KIT (16.6%), followed by TET2 (14.6%), and NRAS (14.6%). The median survival time of 118 patients was 4 months (range, 1-51 months), while the median survival time of 11 patients who received allogeneic hematopoietic stem cell transplantation (allo-HSCT) was 19 months (range, 8-51 months). 4 (36.4%) of the 11 patients experienced relapse within 1 year after transplantation. 1 patient died from a severe infection. Of the 6 surviving patients, 5 patients have received maintenance treatment with decitabine after transplantation, and all remained in a state of recurrence-free survival. Patients with myeloid sarcoma have a very unfavorable outcome. Allo-HSCT is an effective treatment option. Recurrence remains the main cause of transplant failure. Maintenance treatment with decitabine after transplantation can prolong the recurrence-free survival time, although these results must be verified in a study with expanded sample size.

Figure 1

The genetic mutation profile in 41 myeloid sarcoma patients.

Figure 2

(a) Overall survival (OS) according to the myeloid sarcoma subtype: primary myeloid sarcoma (MS, n = 48), myeloid sarcoma with intramedullary disease (n = 51) and relapsed myeloid sarcoma (n = 19). (b) OS according to therapy: local treatment (n = 31), chemotherapy (n = 76), and allogenic hematopoietic stem cell transplantation (allo-HSCT, n = 11).