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Gohring G.,Hannover Medical School | Giagounidis A.,Oncology and Clinical Immunology | Busche G.,Hannover Medical School | Hofmann W.,Hannover Medical School | And 4 more authors.

In patients with low and intermediate risk myelodysplastic syndrome and deletion 5q (del(5q)) treated with lenalidomide, monitoring of cytogenetic response is mandatory, since patients without cytogenetic response have a significantly increased risk of progression. Therefore, we have reviewed cytogenetic data of 302 patients. Patients were analyzed by karyotyping and fluorescence in situ hybridization. In 85 patients, del(5q) was only detected by karyotyping. In 8 patients undergoing karyotypic evolution, the del(5q) and additional chromosomal aberrations were only detected by karyotyping. In 3 patients, del(5q) was only detected by fluorescence in situ hybridization, but not by karyotyping due to a low number of metaphases. Karyotyping was significantly more sensitive than fluorescence in situ hybridization in detecting the del(5q) clone. In conclusion, to optimize therapy control of myelodysplastic syndrome patients with del(5q) treated with lenalidomide and to identify cytogenetic non-response or progression as early as possible, fluorescence in situ hybridization alone is inadequate for evaluation. Karyotyping must be performed to optimally evaluate response. © 2011 Ferrata Storti Foundation. Source

Neukirchen J.,Heinrich Heine University Dusseldorf | Schoonen W.M.,Amgen | Strupp C.,Heinrich Heine University Dusseldorf | Gattermann N.,Heinrich Heine University Dusseldorf | And 3 more authors.
Leukemia Research

Population-based data on patients with MDS are scarce. Here we report the incidence and prevalence of MDS based on data from the Düsseldorf MDS Registry. Cases in the city of Düsseldorf in the study period were identified from the MDS Registry. We calculated crude, calendar-year, age- and sex-specific and European Standard Population age-standardized incidence rates as well as point prevalences per 100,000 The crude incidence rate was 4.15/100,000/year and the point prevalence per 100,000 persons of 7. We found that the incidence and prevalence of MDS was higher in men than women and increased sharply with increasing age. © 2011 Elsevier Ltd. Source

Neukirchen J.,Heinrich Heine University Dusseldorf | Nachtkamp K.,Heinrich Heine University Dusseldorf | Schemenau J.,Heinrich Heine University Dusseldorf | Aul C.,Oncology and Clinical Immunology | And 6 more authors.
Leukemia Research

During the last years, more and more treatment modalities are available for MDS patients. Therefore, we were interested if this is reflected in an improvement of the outcome of the patients. We analyzed the survival and rate of leukemic progression of 4147 patients from the Duesseldorf MDS registry diagnosed during the last 30 years and found an improvement of survival in those patients diagnosed after 2002 (30 vs. 23 months, p < 0.0001). In detail, the improvement of the prognosis was restricted to high-risk MDS patients diagnosed between 2002 and 2014 in comparison to the patient group diagnosed between 1982 and 2001 (19 vs. 13 months, p < 0.001), whereas the prognosis of low-risk MDS patients did not change significantly. The improvement of survival was still measurable after exclusion of RAEB-t patients and of those, that received an allogeneic stem cell transplantation. In line with this finding, we found a lower AML progression rate in the later diagnosed group. Unfortunately, we could not identify a clear reason for this finding but rather a multifactorial cause should be assumed. As death due to bleeding complications and infections was significantly lower, an improvement of BSC may be one of the underlying causes. © 2015 Elsevier Ltd. Source

Gattermann N.,Oncology and Clinical Immunology | Kndgen A.,Oncology and Clinical Immunology | Kellermann L.,Oncology Information Service | Zeffel M.,Celgene | And 2 more authors.

Background: Myelodysplastic syndromes (MDS) are a heterogenous group of clonal hematopoietic stem cell disorders. Patients and Methods: In order to assess current diagnosis and treatment patterns in Germany, the data of 269 patients with MDS from 57 representative centers were analyzed. Results: The most common symptom leading to an initial diagnosis of MDS was anemia (79%). WHO classification, cytogenetic analysis, and IPSS scoring were performed in 92, 67, and 61% of patients, respectively. 5q deletions were identified in 34% of patients whose cytogenetic status was analyzed. Symptomatic anemia was the major trigger for initiating therapy. 49% of patients received supportive care only, and 49% received active therapy (i.e., chemo-, immunomodulatory, or epigenetic therapy), including 5% who received allogeneic transplantation. Of those patients treated with active therapy, approximately half of the higher-risk patients received azacitidine, and approximately half of the lower-risk patients received lenalidomide. Overall, 80% of patients received some form of supportive care, mainly red blood cell transfusions. Conclusion: While the WHO classification system is widely used in clinical practice, karyotyping and IPSS risk assessment do not seem to be common standard. Despite encouraging data on the use of effective and novel drugs, such as lenalidomide and azacitidine in MDS therapies, management of the disease could be further improved by more widespread use of risk stratification of patients using cytogenetics and IPSS assessment. © 2012 S. Karger GmbH, Freiburg. Source

Germing U.,Heinrich Heine University Dusseldorf | Lauseker M.,Ludwig Maximilians University of Munich | Hildebrandt B.,Heinrich Heine University Dusseldorf | Symeonidis A.,University of Patras | And 28 more authors.

Myelodysplastic syndromes (MDS) with del(5q) are considered to have a benign course of the disease. In order to address the issue of the propensity of those patients to progress to acute myeloid leukemia (AML), data on 381 untreated patients with MDS and del(5q) characterized by low or intermediate I International Prognostic Scoring System (IPSS) risk score were collected from nine centers and registries. Median survival of the entire group was 74 months. Transfusion-dependent patients had a median survival of 44 months vs 97 months for transfusion-independent patients (P>0.0001). Transfusion need at diagnosis was the most important patient characteristic for survival. Of the 381 patients, 48 (12.6%) progressed to AML. The cumulative progression rate calculated using the Kaplan-Meier method was 4.9% at 2 years and 17.6% at 5 years. Factors associated with the risk of AML transformation were high-risk World Health Organization adapted Prognostic Scoring System (WPSS) score, marrow blast count >5% and red-cell transfusion dependency at diagnosis. In conclusion, patients with MDS and del(5q) are facing a considerable risk of AML transformation. More detailed cytogenetic and molecular studies may help to identify the patients at risk of progression. © 2012 Macmillan Publishers Limited. Source

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