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Updated: Nov 1, 2025
Use of Hematopoietic Stem Cell Transplantation to Assess the Origin of Myelodysplastic Syndrome
Published on: October 3, 2018
Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia
Huaquan Wang1, Haiyue Niu1, Tian Zhang1
1Department of Hematology, General Hospital, Tianjin Medical University, Tianjin, People's Republic of China.
Objectives:
Our aim is to investigate the clinical characteristics of low- and intermediate-risk myelodysplastic syndrome (MDS) with pure red cell aplasia (PRCA).
Methods:
We retrospectively reviewed the patients of low- and intermediate-risk MDS patients who had been diagnosed with PRCA in our hospital between January 2010 and December 2019.
Results:
There were 6 low- and intermediate-risk MDS patients with PRCA in our study, 1 male and 5 females, with a median age of 63.5 (50-75) years. It accounted for 7.7% (6/78) of all diagnosed PRCA cases and 1.67% (6/359) of diagnosed MDS cases during the same period. All patients were treated with multiple drugs, including recombinant human erythropoietin, cyclosporine, glucocorticoids, androgen, sirolimus, intravenous immunoglobulin and decitabine. Two patients achieved complete remission, two patients achieved partial remission and became blood transfusion independent. Two patients had no response and one patient died.
Conclusion:
Low- and intermediate-risk MDS with PRCA was difficult to treat, but the prognosis was good.
Insights
This study examined low- and intermediate-risk myelodysplastic syndromes (MDS) with pure red cell aplasia (PRCA). While challenging to treat, these cases showed a favorable prognosis with multi-drug therapy.
Area of Science:
- Hematology
- Oncology
Background:
- Myelodysplastic syndromes (MDS) are a group of clonal hematopoietic stem cell disorders.
- Pure red cell aplasia (PRCA) is a rare complication characterized by the selective absence of erythroid precursors in the bone marrow.
- The clinical characteristics and treatment outcomes of MDS with PRCA are not well-defined.
Purpose of the Study:
- To investigate the clinical features of low- and intermediate-risk myelodysplastic syndrome (MDS) patients who also present with pure red cell aplasia (PRCA).
- To evaluate the treatment responses and prognosis of this specific patient cohort.
Main Methods:
- Retrospective review of medical records.
- Inclusion criteria: patients diagnosed with low- or intermediate-risk MDS and PRCA between January 2010 and December 2019.
- Data collected on patient demographics, treatments administered, and clinical outcomes.
Main Results:
- Six patients with low- and intermediate-risk MDS and PRCA were identified (1 male, 5 females; median age 63.5 years).
- These cases represented 7.7% of PRCA diagnoses and 1.67% of MDS diagnoses during the study period.
- Multi-drug therapy (including erythropoietin, cyclosporine, glucocorticoids, etc.) resulted in complete remission in 2 patients, partial remission in 2, no response in 2, and 1 death.
Conclusions:
- Low- and intermediate-risk MDS with PRCA presents a complex clinical challenge.
- Despite treatment difficulties, a good prognosis can be achieved with appropriate multi-agent therapeutic strategies.
- Further research is warranted to optimize treatment protocols for this rare condition.
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