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Database-guided Flow-cytometry for Evaluation of Bone Marrow Myeloid Cell Maturation
Published on: November 3, 2018
[The 511th case: severe anemia with increased ringed sideroblasts]
1Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Abstract:
This report describes the case of a 43-year-old male presenting with severe anemia (hemoglobin, HGB 35 g/L). A comprehensive evaluation encompassing bone marrow morphology (24% ring sideroblasts), genetic testing (SF3B1-negative), iron metabolism studies (ferritin 1 179 μg/L), and imaging (liver MRI T2* value 1.1 ms) was performed for diagnosis, treatment planning, and assessment of outcomes. Initially diagnosed as having myelodysplastic syndrome with ring sideroblasts (MDS-RS), the patient experienced relapse after 10 months of luspatercept treatment. Following the identification of a significant retrospective history of heavy alcohol consumption (250 g/day for 20 years), vitamin B6 therapy resulted in a rapid increase in HGB to 85 g/L within 10 days, reaching 134 g/L after one month, confirming a diagnosis of alcohol-induced sideroblastic anemia (SA). This case highlights that in SA lacking clonal evidence, the exclusion of reversible causes such as alcoholism should be prioritized. While luspatercept demonstrated short-term efficacy in this patient with non-clonal SA, caution is warranted regarding potential masking of the underlying etiology. Concurrent monitoring of iron overload and early initiation of iron chelation therapy are crucial in alcoholic SA to prevent hepatic damage.
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