Diagnosis and Therapy of Myelodysplastic Syndromes in Germany: A Retrospective Multicenter Analysis

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.

[1]  A. Hutchings,et al.  A review of anemia as a cardiovascular risk factor in patients with myelodysplastic syndromes. , 2011, American journal of blood research.

[2]  T. Haferlach,et al.  Meeting report: Vienna 2008 Workshop of the German–Austrian Working Group for Studying Prognostic Factors in Myelodysplastic Syndromes , 2009, Annals of Hematology.

[3]  H. Kantarjian,et al.  Characteristics of US patients with myelodysplastic syndromes: results of six cross-sectional physician surveys. , 2008, Journal of the National Cancer Institute.

[4]  D. Haase Cytogenetic features in myelodysplastic syndromes , 2008, Annals of Hematology.

[5]  M. Gordon Lenalidomide in the Myelodysplastic Syndrome with Chromosome 5q Deletion , 2008 .

[6]  Thomas H Müller,et al.  New insights into the prognostic impact of the karyotype in MDS and correlation with subtypes: evidence from a core dataset of 2124 patients. , 2007, Blood.

[7]  G. Mufti,et al.  CDKN2B methylation status and isolated chromosome 7 abnormalities predict responses to treatment with 5-azacytidine , 2007, Leukemia.

[8]  M. Cazzola,et al.  Time-dependent prognostic scoring system for predicting survival and leukemic evolution in myelodysplastic syndromes. , 2007, Journal of clinical oncology : official journal of the American Society of Clinical Oncology.

[9]  R. Claus,et al.  Preferential cytogenetic response to continuous intravenous low-dose decitabine (DAC) administration in myelodysplastic syndrome with monosomy 7. , 2007, Blood.

[10]  U. Germing,et al.  Treatment of patients with high-risk myelodysplastic syndromes , 2007 .

[11]  M. Cazzola,et al.  Myelodysplastic syndromes--coping with ineffective hematopoiesis. , 2005, The New England journal of medicine.

[12]  G. Mufti Pathobiology, classification, and diagnosis of myelodysplastic syndrome. , 2004, Best practice & research. Clinical haematology.

[13]  G. Mufti,et al.  Guidelines for the diagnosis and therapy of adult myelodysplastic syndromes , 2003, British journal of haematology.

[14]  A. Ganser,et al.  Evaluating the prognosis of patients with myelodysplastic syndromes , 2002, Annals of Hematology.

[15]  U. Germing,et al.  Validation of the WHO proposals for a new classification of primary myelodysplastic syndromes: a retrospective analysis of 1600 patients. , 2000, Leukemia research.

[16]  T Hamblin,et al.  International scoring system for evaluating prognosis in myelodysplastic syndromes. , 1997, Blood.

[17]  H. Gralnick,et al.  Proposals for the classification of the myelodysplastic syndromes , 1982, British journal of haematology.