Myelodysplastic syndrome/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis: 4 cases
10.3760/cma.j.cn115356-20230222-00046
- VernacularTitle:骨髓增生异常综合征/骨髓增殖性肿瘤伴环形铁粒幼细胞和血小板增多4例
- Author:
Juanjuan XIAO
1
;
Shaojie YE
;
Huimei GUO
;
Songying ZHAO
;
Jing WANG
;
Hua XUE
Author Information
1. 河北大学附属医院血液科，保定 071000
- Keywords:
Myelodysplastic-myeloproliferative diseases;
Anemia, sideroblastic;
Thrombocytosis
- From:
Journal of Leukemia & Lymphoma
2024;33(6):352-356
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To enhance the understanding of the diagnosis and individualized treatment of myelodysplastic syndrome/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T).Methods:A retrospective case series study was conducted. The clinical data, diagnosis and treatment process and prognosis of 4 patients with MDS/MPN-RS-T admitted to Affiliated Hospital of Hebei University from September 2015 to May 2021 were retrospectively analyzed, and the related literature was reviewed.Results:All the 4 patients were male, aged 63 to 75 years. Patients 1 and 2 were classified as revised international prognostic scoring system (IPSS-R) high-risk group, combined with ASXL1 mutation and high risk cytogenetic abnormality. The therapeutic effect of various treatment regimens was poor, and they were converted to acute myeloid leukemia (AML) and then died due to disease progression. Patient 3 was classified as IPSS-R medium-risk group. His main manifestation was myelodysplastic syndrome (MDS) combined with ring sideroblasts in the early stage and was transformed into MDS/MPN-RS-T during the treatment, and JAK2 mutation occurred in the subsequent treatment. After lenalidomide treatment, the patient was removed from blood transfusion and the condition was stable at present. Patient 4 was classified as IPSS-R medium-risk group, and lenalidomide showed significant therapeutic effects and he was in stable condition.Conclusions:Lenalidomide can significantly improve transfusion dependence in patients with MDS/MPN-RS-T, and ASXL1 mutation and high-risk cytogenetic abnormality may be associated with AML transformation.
