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1356 Part X: Malignant Myeloid Diseases Chapter 87: Myelodysplastic Syndromes 1357

TABLE 87–5. Survival of Patients Based on the International Prognostic Scoring System
IPSS Group at Diagnosis No. of Patients 2-Year Survival 5-Year Survival 10-Year Survival 15-Year Survival
Low 267 85% 55% 28% 20%
Intermediate-1 314 70% 35% 17% 12%
Intermediate-2 179 30% 8% 0 –
High 56 5% 0 – –

IPSS, International Prognostic Scoring System.

clinic use even though these lesions have been shown to have indepen- agents, cytotoxic agents, or AHSCT is typically reserved for higher-risk
dent prognostic significance. Future prognostic models are likely to patients. 305–307 In addition, two biomarkers—the presence of del(5q) in
combine clinical and molecular information to refine the prediction of lower-risk patients with anemia, and the serum erythropoietin level in
prognosis in MDS. anemic patients—are strong enough predictors of treatment response
as to permit selection or avoidance of individual drugs (i.e., the use of
TREATMENT OF MYELODYSPLASTIC lenalidomide or erythropoiesis-stimulating agents, respectively).
308
309
SYNDROMES BASED ON PROGNOSTIC While other predictive biomarkers in MDS have been proposed, such
as TET2 and DNMT3A mutation status as predictors of the likelihood
SCORE of response to hypomethylating agents 194,310,311 ; patient age, marrow
hypocellularity or human leukocyte antigen (HLA) DR-B15 status and
Therapeutic decisions in patients with MDS patients can be based on response to immunosuppressive therapy ; serum thrombopoietin and
312
the predicted evolution of disease, as measured by prognostic tools intensity of platelet transfusion requirements and response to thrombo-
304
such as the IPSS and IPSS-R. Lower-risk patients are commonly poietin agonist romiplostim ; or flow cytometry patterns and response
313
treated with lower-intensity therapies such as hematopoietic growth to erythropoiesis-stimulating agents , these are not strong enough
314
factors or immunomodulatory agents, while use of hypomethylating predictors of response to specific therapies to influence management
TABLE 87–6. Revised International Prognostic Scoring System for Myelodysplastic Syndromes 8
Cytogenetic Groups IPSS-R Karyotype Abnormalities
Very good del(11q), –Y
Good Normal, del(20q), del(5q) alone or with 1 other anomaly, del(12p)
Intermediate +8, del(7q), i(17q), +19, +21, any single or double abnormality not listed, or two or more independent clones
Poor der(3q), –7, double with del(7q), complex with 3 abnormalities
Very poor Complex with >3 abnormalities
IPSS-R Parameter Categories and Associated Scores
Very Good Good Intermediate Poor Very Poor
Cytogenetic Risk Group 0 1 2 3 4
Marrow blast % ≤2 >2–<5 5–10 >10
0 1 2 3
Hemoglobin (g/dL) ≥10 8–<10 <8
0 1 1.5
Platelet count (× 10 /L) ≥100 50–<100 <50
9
0 0.5 1
Neutrophil count (× 10 /L) ≥0.8 <0.8
9
0 0.5
Median Survival, 25% with AML,
IPSS-R Risk Group Total Score % of Patients Years at Years
Very low ≤1.5 19 8.8 NR
Low >1.5–3 38 5.3 10.8
Intermediate >3–4.5 20 3 3.2
High >4.5–6 13 1.6 1.4
Very high >6 10 0.8 0.73

AML, acute myelogenous leukemia; IPSS-R, International Prognostic Scoring System–Revised; NR, not reached.
These data were found in Ref 298 and reiterated in this table.

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