Pathy's Principles and Practice of Geriatric Medicine. Группа авторов

Pathy's Principles and Practice of Geriatric Medicine - Группа авторов


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based on the sum of scores for each variable. The usefulness of the IPSS is limited by the fact that patients with secondary MDS were excluded from the analysis, and the information only pertains to patients at the time of diagnosis and cannot be used to estimate the real‐time risk for patients. Furthermore, it may underestimate the impact of cytogenetics.

      Source: Adapted from Greenberg et al.6

Risk category Score Median survival (years) in the absence of therapy 25% AML progression (years) in the absence of therapy
Low 0 5.7 9.4
Intermediate‐1 0.5–1.0 3.5 3.3
Intermediate‐2 1.5–2.0 1.1 1.1
High 2.5 0.4 0.2

      Source: Adapted from Hong et al.44

Prognostic groups Chromosomal categories Median survival time (months)
Very good del(11q), −Y 60.8
Good Normal, del(5q), double aberrations including del(5q), del(12p), del(20q) 48.5
Intermediate del(7q), +8, i(17q), +19, any other, independent clones 25.0
Poor inv(3)/t(3q)/del(3q), −7, −7/7q, double aberrations including −7/7q−, complex karyotypes with 3 abnormalities 15.0
Very poor Complex karyotypes with >3 abnormalities 5.7

      To overcome some of the limitations of the IPSS, which excludes patients with therapy‐related myelodysplastic syndrome, proliferative chronic myelomonocytic leukaemia, and those who have received prior therapy, a group at the University of Texas M.D. Anderson Cancer Center developed a new prognostic scoring system (MDACC) that included all of these patients.61 This model identified four prognostic groups based on a prognostic score that included age, performance status, platelet count, haemoglobin, white blood cell count, bone marrow blast percentage, complex karyotype, and chromosome 7 abnormality and prior transfusions.

      In an attempt to incorporate dynamic variables such as transfusion burden, the WHO prognostic scoring system (WPSS), based on WHO 2008 categories, was developed.62 This model takes into account WHO subgroups, karyotype, and transfusion requirement to classify patients into five risk groups with variable median survivals (12–103 months) and probability of leukaemia conversion. The advantage of the WPSS is that it is a dynamic prognostic scoring system that can be used for patients at any time during the course of their disease.

      A retrospective study by Fega et al. demonstrates that there are other significant predictors of survival in older adults. Low serum albumin, Charlson score, performance status, ability to take a long walk, and interference of physical symptoms in family life are significant predictors to consider.63 In the study, the multivariate model that best predicted mortality included low serum albumin, therapy‐related MDS, IPSS score, and ease taking a long walk.

      Management of MDS comprises supportive care and disease‐modifying agents. Supportive should be given to all patients, while high‐risk patients may be offered active treatments ranging from hypomethylating agents to intensive chemotherapy and haematopoietic stem cell transplantation. Recently, new agents have been proposed for treatments of low‐risk disease.

      Treatment choice in older adults should not be based only on chronological age but should also take into consideration haematological variables (IPSS score, genetic risk) as well as variables derived from a CGA (comorbidity burden, self‐sufficiency, frailty status) and life expectancy. Patient preferences should always be taken into account.

      Treatment of lower‐risk MDS

      Supportive measures for anaemia: erythroid‐stimulating agents

      Erythroid‐stimulating agents (ESAs) are commonly used to treat MDS patients to reduce transfusion requirements. The use of ESAs in MDS is supported by the American Society of Hematology (ASH), the American Society of Clinical Oncology (ASCO), and the National Comprehensive Cancer Network (NCCN).68,69

      Two large phase 3 studies investigated the role of ESA in treating anaemia in MDS patients.70,71 Based on the results of these randomized controlled trials (RCTs), which showed a lower response rate for darbepoetin compared to epoetin‐alfa (14.7 versus 31.8%), EMA approved the use of the second one for this indication.

      Source: Adaped from Buckstein et al.67

Nordic score ITACA score
Variables (1 point each):Transfusions >2 units
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