Guidelines for diagnosis and management of Adult Myelodysplastic Syndromes (MDS)

Transcription

1 Guidelines for diagnosis and management of Adult Myelodysplastic Syndromes (MDS) Author: Dr A Pillai, Consultant Haematologist On behalf of the Haematology CNG Re- Written: February 2011, Version 2 Revised: December 2016, Version 3 Review: No later than December 2018 MCCN guidelines - Adult Myelodysplastic Syndromes December 2016 Page 1 of 7

2 Introduction Myelodysplastic syndromes (MDS) are a group of myeloid neoplasms predominating in the elderly, characterised by dysplastic changes in one or more cell lineages, ineffective haematopoiesis and a variable predilection to development of acute myeloid leukaemia. Investigations The diagnosis of myelodysplastic syndromes involves the careful examination of well-stained blood films and bone marrow aspirate smears, together with an iron stain and a bone marrow trephine specimen. The bone marrow investigations should be undertaken as per the MCCN - HODS diagnostic pathways. MCCN guidelines - Adult Myelodysplastic Syndromes December 2016 Page 2 of 7

3 Diagnosis and Classification As MDS are clonal haematopoietic disorders, the diagnosis is uncertain in the absence of a cytogenetic/fish/molecular lesion and no excess of blasts, especially if there is no convincing dysplasia in the bone marrow. The diagnosis and classification of MDS should be based on the World Health Organization Classification (WHO, 2016 revision) (Arber et al, 2016), which has superseded the former (WHO, 2008 revision, Swerdlow et al, 2008) and French-American-British (FAB) Classification (Bennett et al, 1982). Risk Stratification The IPSS-R is an important tool for assessing the outcome of patients with untreated, primary adult MDS (Greenberg et al, 2012). The most important addition is the newer cytogenetic grouping that gives more accurate prognostic information and helps guide individualised patient management. MCCN guidelines - Adult Myelodysplastic Syndromes December 2016 Page 3 of 7

4 Treatment All patients should be discussed at local multi-disciplinary team meeting (MDT) prior to initiating treatment. Clinical teams are also encouraged to enter patients into available NCRN/MRC trials at diagnosis and relapse. Algorithm for Management of low risk MDS MCCN guidelines - Adult Myelodysplastic Syndromes December 2016 Page 4 of 7

5 Predicting response to Erythropoeitin stimulating agent (ESA) The model for predicting response to ESA shown below should be used. Score 0 74%, Score 1 23 %, Score 2 7%. (Hellstrom-Lindberg et al 2003, Jadersten et al 2005). Transfusion need Score S EPO levels Score < 2 units RBC/month 0 < 500 u/l 0 > 2units RBC/month 1 >500 u/l 1 RBC Red blood cell, EPO Erythropoeitin Algorithm for Management of high risk MDS MCCN guidelines - Adult Myelodysplastic Syndromes December 2016 Page 5 of 7

6 Iron chelation 1) Iron chelation therapy cannot be routinely recommended for MDS patients with transfusion iron overload but may be considered in patients with a very good prognosis. 2) Triggers include more than 20 units of red cells transfused or a serum ferritin >1000 g/l and when ongoing red cell support is expected. 3) Desferrioxamine remains the therapy of choice with the longest record of safety and efficacy. Deferasirox (Exjade) is recommended for patients intolerant of desferrioxamine. Deferiprone could be considered in patients with normal baseline neutrophil counts. Supportive care This includes blood product transfusion support with packed red cells and platelets. Transfusion thresholds should be in line with BCSH guidelines. For severely neutropenic patients preventive measures against infection (e.g. during invasive procedures), or, more importantly, rapid onset of broad spectrum antibiotics in case of fever or symptoms of infection is advised in accordance with local protocols. Psychosocial support All patients should be 1) Given contact numbers for a local key worker. 2) Fully counselled as to the nature of the disease and management of their individual situation 3) Offered written information on their disease and given 24 hour direct contact numbers for haematology service. 4) Given information and contact numbers for local and national MDS support groups. Follow up points 1. Risk stratify low risk MDS patients using IPSS-R should there be any deterioration in their cytopenias. 2. Bone marrow examination is generally triggered by worsening of cytopenias or appearance of circulating blasts rather than at regular intervals MCCN guidelines - Adult Myelodysplastic Syndromes December 2016 Page 6 of 7

7 References 1. Arber DA et al, (2016) The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood,127, Greenberg, PL et al, (2012) Revised international prognostic scoringsystem for myelodysplastic syndromes.blood, 120, Fenaux, P et al, (2009) Efficacy of azacitidine compared with that of conventional care regimens in the treatment of higher-risk myelodysplastic syndromes: a randomised, open-label, phase III study. The Lancet Oncology, 10, Hellstrom-Lindberg E et al, (2003) A validated decision model for treating the anaemia of myelodysplastic syndromes with erythropoietin + granulocyte colony-stimulating factor: significant effects on quality of life. British Journal of Haematology, 120, Jadersten, M et al, (2005) Long-term outcome of treatment of anemia in MDS with erythropoietin and G-CSF. Blood, 106, Killick SB et al, (2014) Guidelines for the diagnosis and management of adult myelodysplastic syndromes British Journal of Haematology,164, Malcovati L et al, (2013) Diagnosis and treatment of primary myelodysplastic syndromes in adults: recommendations from the European Leukemia Net Blood, 122, Fenaux P et al, (2014) Myelodysplastic syndromes: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology 25, Technology appraisal guidance [TA218] NICE guidance March 2011 Azacitidine for the treatment of myelodysplastic syndromes, chronic myelomonocytic leukaemia and acute myeloid leukaemia. 10. Technology appraisal guidance [TA322] NICE guidance September 2014 Lenalidomide for treating myelodysplastic syndromes associated with an isolated deletion 5q cytogenetic abnormality MCCN guidelines - Adult Myelodysplastic Syndromes December 2016 Page 7 of 7