| BackgroundMyeloid Sarcoma,also known as granulocytic sarcoma or chloroma,is a rare extramedullary lesion,which is formed by aggregation of malignant hematological tumor cells outside the medulla..Burns first described its pathological features in 1811.Because immature medullary precursor cells contained peroxidase particles,which means that the tumor may become green after air exposure.,so it was called chloroma.Its diagnosis went through granulocytic sarcoma and extramedullary myeloid tumors,which were officially named as Myeloid Sarcoma by WHO in 2001 and became a subtype of acute myeloid leukemia in 2009.Myeloid Sarcoma is associated with myeloid leukemia cells.It occurs frequently in patients with acute myeloid leukemia and occasionally in chronic myeloid leukemia,myelodysplastic syndrome and other myeloproliferative diseases.The European Society for Hematology recognizes a variety of extramedullary manifestations of myeloid neoplasms: 1.MS without previous or concurrent AML or other myeloid neoplasm.2.Concurrent MS and AML with a focus on karyotypic or molecular findings.3.MS and AML without recurrent genetic abnormalities.4.MS as a form of blast transformation in a patient with MPN and/or MDS.If there is no evidence of myeloid diseases in bone marrow examination and biopsy within 30 days after MS onset,there is no history of myeloid disease,only localized masses are present,and physical examination,imaging and laboratory examination officially extramedullary is the only tumor site,which is called primary MS.Primary MS is rare,with an incidence of about two millionths.Most patients have bone marrow invasion within months or years.Early diagnosis and treatment are very important.However,the lack of specific laboratory diagnostic evidence and non-specific clinical manifestations often makes the diagnosis of MS difficult,leading to a higher rate of misdiagnosis.In terms of prognosis,literature shows that MS often has poor prognosis.Several retrospective analyses showed that the hematological response rate and OS of patients with MS were significantly lower than those without MS.Because of its complex and diverse clinical manifestations,most of the literature reports are retrospective analysis of individual cases or small samples,and its treatment needs to take into account both the location and size of the mass,its relationship with tissue,patient age and disease status.At present,there is no standard treatment method,and it is urgent to seek a treatment method that can improve the adverse prognosis of patients and prolong the survival of patients.ObjectiveTo observe and summarize the clinical characteristics of myeloid Sarcoma,compare the effects of different treatment methods,analyze the prognostic factors of it,and provide guidance for clinical diagnosis and treatment.MethodsA retrospective analysis was made of 97 MS patients diagnosed in the First Affiliated Hospital of Zhengzhou University from January 1,2010 to May 1,2019.The clinical data of gender,age,location of onset,immunohistochemistry,diagnose,treatment,follow-up time and outcome were analyzed,and the risk factors affecting prognosis were analyzed.Result1.97 patients with MS,including 57 males and 40 females,the ratio of male to female patients was 1.43:1,and the median age of onset was 46(1~81)years old.The most common site of infection was soft tissue,accounting for 30.2%(32/106);followed by lymph nodes,accounting for 25.5%(27/106);male and female reproductive system(cervix,ovary,testis,epididymis,spermatic cord)accounted for 10.3%(11/ 106);also occurs in the eyes,spleen,stomach,esophagus,etc,but less common.2.MPO has the highest expression rate in immunohistochemistry of the 97 patients,which is 87.5%(84/96);CD43 expression rate is 86.7%(52/60);CD117 expression rate is 65.2%(30/46));CD34 expression rate was 51.4%(19/37);CD123 expression rate was 29.2%(7/24).The lymphocyte lineage markers were also expressed,with CD7 expression rate of 41.9%(13/31),CD5 expression rate of 40%(4/10),TdT expression rate of 31.7%(20/63),and CD3 expression rate of 30.1%(28/93).The expression rate of CD79 a was 27.7%(13/47);the expression rate of CD20 was 13.8%(13/94);the expression rate of CD10 was 10.8%(4/37).The median Ki-67 index is 60%(10%~90%).3.Of the 97 patients,21 cases underwent karyotype analysis,of which 8(38.1%)cases were abnormal,and 4 of them had t(8;21)translocation.Twenty-one patients underwent screening for myeloid gene mutations,and 13 of them(61.9%)were abnormal.4.25 cases were treated with local treatment alone in the 89 patients,with a median follow-up time of 1(1~4)months.56 patients underwent chemotherapy combined with local treatment.The median follow-up time was 4(1~60)months.Allogeneic hematopoietic stem cell transplantation was performed after induction complete remission of 8 cases,whose median follow-up time was 15.5(8~24)months.Two of them were treated with decitabine after transplantation and were followed up to 10 months?20 months and 24 months after diagnosis,respectively.5.The median onset time of MS after hematopoietic stem cell transplantation was 36(14-38)months.6.There was no significant difference in gender,age,Ki-67 index,single-site and multiple-site?chromosome abnormalities?the expression of lymphocyte lineage markers.The P values were 0.422,0.713,0.479,0.316 and 0.064,respectively.Chromosome prognosis stratification and the expression of T/B cell markers had no significant effect on the prognosis.The P values were 0.227 and 0.326.Diagnosis and treatment on the prognosis was statistically different.The P values were 0.013,0.003.Chemotherapy combined with hematopoietic stem cell transplantation have long median survival time.The survival time of primary MS is shorter.Cox survival analysis was performed with follow-up time as the dependent variable and diagnosis and treatment as independent variables,indicating that there was a statistically significant association between the treatment regimen and clinical outcome.Conclusion1.The site of MS is not specific.The survival time is short and the median survival time is 5 months.2.MS secondary to transplantation usually occurs one year after transplantation,which needs to be vigilant.3.Gender,age,single or multiple,Ki-67 index,the expression of T/B cell markers did not affect the prognosis of MS.Chromosomal abnormalities and stratification may not affect the prognosis of MS,but the number of cases is small,which needs to be further confirmed by increasing the sample size.4.Chemotherapy combined with hematopoietic stem cell transplantation is an effective treatment for MS.Recurrence is the main factor affecting the outcome of transplantation.Decitabine may play an important role in the maintenance of MS after transplantation,but it still needs a large sample of clinical trials to confirm. |
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