krebsmedizin

Editorial: "Myelodysplastische Syndrome"

 

Von A. Giagounidis

"Molecular Changes in Myelodysplastic Syndromes – Diagnostic Implications"

 

The FAB classification of MDS, which was established in 1982, was based on morphological changes and the number of bone marrow blasts in patients with MDS. This classification was supplemented by the impact of a number of recent detected chromosomal changes which are specific for MDS. Their findings correlated distinct diagnostic and prognostic subtypes of this heterogenous disease. For example, a wide range of different aberrations have been described in MDS such as loss or multiplication of genetic material and structural alterations. However, in only 50 % of MDS cases conventional cytogenetic testing can detect such aberrations.

Von W.-K. Hofmann

In recent years, new treatment options for MDS have been developed, like epigenetic treatment with demethylating agents, or immunomodulatory treatment with lenalidomide. However, many patients will ultimately need chronic transfusion therapy. About 80 % of MDS patients have a hemoglobin of less than 10 g/dl at the time of diagnosis, and the majority of these patients become transfusion dependent. As every unit of blood transfused provides 200 to 250 mg of iron and normal daily losses are only about 1 to 2 mg, iron overload is unavoidable with chronic transfusion therapy. Besides this excess of iron from chronic transfusion, MDS patients start accumulating iron even before they receive their first blood transfusion.

 

Von N. Gattermann

"Assessing the Prognosis in Patients with Myelodysplastic Syndromes"

 

Myelodysplastic syndromes (MDS) are characterized by ineffective hematopoiesis, dyplastic features in blood and marrow and an increased risk of acute myeloid leukemia (AML) evolution. The clinical course for MDS is variable and focuses on morphology type and prognostic parameters. Although MDS classification following WHO (World Health Organization) guidelines provides some prognostic information, considering additional prognostic parameters is important for more precise treatment.

 

Von U. Germing, Kathrin Nachtkamp, Corinna Strupp

"A Short Review on the Use of Demethylating Agents in Myelodysplastic Syndromes"

 

In the treatment of patients with MDS it has become increasingly clear that intensive treatment strategies are not ideal for a large number of patients. This is, of course, due to the fact that most patients are elderly and suffer from significant comorbidities. Additionally, a significant proportion of those patients will also have adverse karyotypic abnormalities that have a negative impact on the ability to achieve complete remission with intensive chemotherapy. Previously, such patients would have been treated with a low-dose chemotherapy, including cytarabine, melphalan and others, or with supportive care only. The demethylating agents 5-azacitidine and decitabine are of interest because they allow the majority of these patients to undergo palliative treatment in an outpatient setting.

 

Von A. Giagounidis, C. Aulnot

"New Insights into the Cytogenetic Heterogeneity of Myelodysplastic Syndromes"

 

Von D. Haase

"Intensive Chemotherapy and Allogeneic Stem Cell Transplantation in MDS Patients – a Question of Patient Selectio"

 

Several therapeutic modalities for patients with myelodysplastic syndromes have been developed over the past few years, and some of these may change the natural course of the disease. With the rare exception of patients who achieve long lasting remissions with allogeneic HSCT is currently the only modality with proven curative potential. This short review will focus on the current developments for both treatment modalities.

Von U. Platzbecker, G. Ehninger

"The Role of Lenalidomide in Myelodysplastic Syndromes"

 

Understanding of the myelodysplastic syndromes (MDS), particularly of the molecular underpinnings of these diseases and of rational therapeutic approaches, has increased exponentially over the past three decades. This is demonstrated particularly clearly when one considers that among the patients included in the international prognostic scoring system (IPSS), published in 1997 and based on data collected across seven registries from the mid-1980s through the mid-1990s, 84 % had received no therapy at all, and cytogenetic risk categorization was based essentially on six abnormalities (5q-, 20q-, -Y, -7/7q-, complex, and “other”) . Now, in the year 2008, three drugs are approved by the Food and Drug Administration (FDA), along with five growth factors that can potentially be used in MDS patients, making it difficult to design clinical trials for the truly de novo population.

 

Von A. Serkeres