NCCN Clinical Practice Guidelines. Myelodysplastic Syndromes
These suggested practice guidelines are based on extensive evaluation of the reviewed risk-based data and indicate useful current approaches for managing patients with MDS. Four drugs have recently been approved by the FDA for treating specific subtypes of MDS: lenalidomide for MDS patients with del(5q) cytogenetic abnormalities; azacytidine and decitabine for treating patients with higher-risk or nonresponsive MDS; and deferasirox for iron chelation of iron overloaded patients with MDS. However, because a substantial proportion of patient subsets with MDS lack effective treatment for their cytopenias or for altering disease natural history, clinical trials with these and other novel therapeutic agents along with supportive care remain the hallmark of management for this disease. The role of thrombopoietic cytokines for management of thrombocytopenia in MDS needs further evaluation. In addition, further determination of the effects of these therapeutic interventions on the patient's quality of life is important.(116,119,120,128,129) Progress toward improving management of MDS has occurred over the past few years, and more advances are anticipated using these guidelines as a framework for coordination of comparative clinical trials.
Available from: Avinash G Dinmohamed
- "Bone marrow examinations are essential for the correct diagnosis of MDS and CMML as recommend by international expert panels    . The diagnoses in the DBC cohort cannot be pathologically confirmed due to the anonymous data provided by the DIS; therefore, we cannot obtain diagnostic information by retrospective medical record reviews. "
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ABSTRACT: Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML) may be underreported in cancer registries such as the Netherlands Cancer Registry (NCR). Analysis of Dutch medical claims can complement NCR data on MDS and CMML. We analyzed data on 3681 MDS patients and 235 CMML patients aged ≥18 years with initial claims for MDS or CMML from the Dutch nationwide medical claims-based Diagnosis Treatment Combination Information System (DIS) between 2008 and 2010. Clinical information was available in the DIS. MDS and CMML were diagnosed without a bone marrow (BM) examination in almost half of the patients. The age-standardized incidence rate (ASR) per 100,000 in the cohort that underwent BM examinations compared with NCR data was 2.8 vs. 3.3 for MDS and 0.2 vs. 0.4 for CMML in 2008–2010. A conservative treatment approach was associated with increasing age and absence of BM examination in MDS (p < 0.001 for both) and CMML patients (p < 0.033 for both). In conclusion, the ASR of MDS in the cohort that underwent BM examinations was comparable with the NCR. The majority of elderly patients, either with or without BM examinations, received no therapy. Together, MDS and CMML may be misdiagnosed and inappropriately managed without a BM confirmation.
Available from: Rebecca Holman
- "It has been shown that comorbidity has a significant impact on the clinical outcome, in addition to other patient-related factors such as age and performance status   . Recent studies showed that comorbidity, age and performance status should have an important role in therapy decision making, since these factors are predictive for the benefit of allogeneic stem cell transplantation   . "
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ABSTRACT: Myelodysplastic syndromes (MDS) comprise bone marrow failure diseases with a diverse clinical outcome. For improved risk stratification, the International Prognostic Scoring System (IPSS) has recently been revised (IPSS-R). This single-centre study aimed to validate the IPSS-R and to evaluate prior prognostic scoring systems for MDS. We retrospectively analysed 363 patients diagnosed with MDS according to the FAB criteria between 2000 and 2012. The IPSS, MD Anderson Risk Model Score (MDAS), World Health Organisation (WHO)-classification based Prognostic Scoring System (WPSS), refined WPSS (WPSS-R), IPSS-R and MDS-Comorbidity Index (MDS-CI) were applied to 222 patients considered with primary MDS following the WHO criteria and their prognostic power was investigated. According to the IPSS-R, 18 (8%), 81 (37%), 50 (23%), 43 (19%) and 30 (13%) patients were classified as very low, low, intermediate, high and very high risk with, respectively, a median overall survival of 96 (95% Confidence interval (CI) not reached), 49 (95% CI 34-64), 22 (95% CI 0-49), 19 (95% CI 11-27) and 10 (95% CI 6-13) months (p<.000). The IPSS-R showed improved prognostic power as compared to the IPSS, MDAS, WPSS and WPSS-R. Furthermore, the MDS-CI refined the risk stratification of MDS patients stratified according to the IPSS-R. In conclusion, accounting for the disease status by means of the IPSS-R and comorbidity through the MDS-CI considerably improves the prognostic assessment in MDS patients.
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Available from: Mark Drummond
- "Several studies purporting to demonstrate improved survival for chelated patients are all retrospective and methodologically limited (Rose et al, 2010). Despite this, there is almost universal recommendation in national and international guidelines for iron chelation therapy in selected MDS patients (Greenberg et al, 2011). 4 Which iron chelation therapy should be used (if any)? "
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ABSTRACT: of key recommendations Diagnosis 1 Myelodysplastic syndrome (MDS) should be suspected in patients with otherwise unexplained cytopenias(s) or mac-rocytosis. Grade 1A 2 The initial assessment of a patient with unexplained cytopenias(s) may not confirm a diagnosis of MDS. Fur-ther follow-up and reassessment may be necessary to reach a firm diagnosis. Grade 2B,C 3 Initial assessment of a patient with suspected MDS should include a minimum set of investigations and the differen-tial diagnosis of marrow dysplasia should be considered. Grade 1A 4 Patients with MDS should be assessed by a haematologist and, except where clearly inappropriate, offered review by a regional or national expert given the disease rarity. 5 All cases of MDS should be classified according to the World Health Organization (WHO) Revised Classification 2008. Grade 1A 6 Bone marrow cytogenetic analysis should be performed on all patients with suspected MDS having a bone marrow examination. Grade 1A 7 Consideration should be given at diagnosis to the progno-sis for each individual patient, with application of the revised International Prognostic Scoring System (IPSS-R). Grade1B 8 All cases of MDS should be reported to the National Cancer Registry and MDS-specific registries if applicable.
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