Clonal architecture of del(5q) myelodysplastic syndromes: aberrant CD5 or CD7 expression within the myeloid progenitor compartment defines a subset with high clonal burden

Oelschlaegel, Uta; Röhnert, Maximilian Alexander; Mohr, Brigitte; Sockel, Katja; Herold, Sylvia; Ehninger, Gerhard; Bornhäuser, Martin; Thiede, Christian; Platzbecker, Uwe

doi:10.1038/leu.2015.158

Cited by 9 publications

(3 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Oelschlaegel et al [17] provided some explanation for the above-mentioned theory of phenotypic alterations and subsequent resistance to ESA on the clonal level. The frequency of clonal cells was significantly higher in a subgroup of predominantly lower-risk MDS harboring del(5q) plus an aberrant CD5/CD7 expression compared with IPSS-R matched patients without this aberrant antigen expression [18]. Moreover, they showed that in anemic lower-risk MDS patients with aberrant CD5/7 expression, slightly higher serum EPO levels may cause the significant lower response rates to ESA therapy irrespective of comparable other clinical predictive markers like transfusion burden [18].…”

Section: Current Standards To Treat Anemia In Low-risk Mdsmentioning

confidence: 99%

“…The frequency of clonal cells was significantly higher in a subgroup of predominantly lower-risk MDS harboring del(5q) plus an aberrant CD5/CD7 expression compared with IPSS-R matched patients without this aberrant antigen expression [18]. Moreover, they showed that in anemic lower-risk MDS patients with aberrant CD5/7 expression, slightly higher serum EPO levels may cause the significant lower response rates to ESA therapy irrespective of comparable other clinical predictive markers like transfusion burden [18]. Rigolin et al detected a higher percentage of cytogenetically abnormal karyotypes in patients nonresponsive to ESA [19].…”

Section: Current Standards To Treat Anemia In Low-risk Mdsmentioning

confidence: 99%

See 1 more Smart Citation

Setting Fire to ESA and EMA Resistance: New Targeted Treatment Options in Lower Risk Myelodysplastic Syndromes

Kubasch

Platzbecker

2019

IJMS

View full text Add to dashboard Cite

During the last decade, substantial advances have been made in the understanding of the complex molecular, immunological and cellular disturbances involved in the initiation as well as evolution of myelodysplastic syndromes (MDS). In 85% of the mainly frail and older patient population, anemia is present at the time of diagnosis and is thus a major therapeutic challenge. High rates of primary resistance to erythropoiesis-stimulating agents (ESAs), the currently only approved standard therapy to treat anemia in lower-risk MDS, demand the development of novel and efficient drugs with a good safety profile. Luspatercept, a ligand trap of activin receptor II, is able to promote late stage erythropoiesis even in patients failing prior ESA treatment. The presence of ring sideroblastic phenotype defines a subgroup of patients with higher response rates. Additionally, recent developments in clinical research using HIF-1 or telomerase modulation by roxadustat or imetelstat are promising. Other areas of translational research involve targeting the inflammasome by anti-inflammatory drugs in order to improve anemia. These efforts will hopefully pave the way for new targeted treatment options for anemic low-risk MDS patients.

show abstract

Section: Current Standards To Treat Anemia In Low-risk Mdsmentioning

confidence: 99%

Section: Current Standards To Treat Anemia In Low-risk Mdsmentioning

confidence: 99%

Setting Fire to ESA and EMA Resistance: New Targeted Treatment Options in Lower Risk Myelodysplastic Syndromes

Kubasch

Platzbecker

2019

IJMS

View full text Add to dashboard Cite

show abstract

“…The 5q-syndrome is the most distinct type of all MDSs as it has clear genotype/phenotype relationship [18,21]. If del (5q) occurs as the only cytogenetic abnormality, it is associated with favorable prognosis but once it is encountered in association with other single or multiple chromosomal abnormalities, particularly in the setting of complex cytogenetics, the clinical outcome is rendered poor [14,16,20,22,23].…”

Section: Del (5q) Type Of Mdsmentioning

confidence: 99%

Myelodysplastic Disorders, 5q-Syndrome

Al-Anazi¹

2016

Myelodysplastic Syndromes

View full text Add to dashboard Cite

The myelodysplastic syndromes (MDSs) are characterized by ineffective erythropoiesis and progressive cytopenia and ultimately affected patients develop acute myeloid leukemia (AML) or die from advanced bone marrow (BM) failure. Myelodysplastic syndrome (MDS) with isolated del (5q) is a common type of MDS with specific pathological and clinical manifestations including refractory anemia. It is usually treated by (1) supportive measures including blood transfusions that may cause iron overload that requires iron chelation therapy, (2) targeted therapies such as the immunomodulatory drug lenalidomide, and (3) hematopoietic stem cell transplantation (HSCT) in transplant eligible individuals. The establishment of the various prognostic systems, the discovery of the new genetic mutations, and the identification of new targets, in MDSs in general and in 5q-syndrome in particular, will hopefully translate into more pinpointed targeted therapies that will further improve the outcomes of patients having these disorders.

show abstract