Table 4. Risk tools in CML.
Risk Tool | Risk Factors | Risk Groups | a) Population, b) Treatment used |
Predictive/Prognostic Implication |
---|---|---|---|---|
Tura et al (1981) [18] | Factors: 1. Splenomegaly > 15 cm below costal margin, 2. Hepatomegaly > 6cm below costal margin 3. Thrombocytopaenia < 50x109/l or thrombocytosis > 500x109/l 4. Leucocytosis >100x109/l 5. Blasts in peripheral blood > 1% 6. Promyelocytes and myelocytes peripheral blood > 20% |
Stage I (low risk): 0–1 factor Stage II (intermediate risk): 2–3 factors Stage III (high risk): 4–6 factors |
a) 255 cases b) Chemotherapy |
OS significantly different between three groups (p <0.0005) |
Cervantes et al (1982) [19] | 1. Splenomegaly 2. Hepatomegaly 3. Erythroid precursors in peripheral blood 4. Myeloblasts in bone marrow > 5% |
Stage I (low risk): 0–1 factor Stage II (intermediate risk): 2 factors Stage III (high risk): 3–4 factors |
a) 121 cases, Spain b) Busulfan |
5 year OS Stage I-70%, Stage II-30% Stage III-15% |
Kantarjian et al (1990) [20] | 1. Age ≥ 60 2. Blasts in peripheral blood ≥3% 3. Blasts in bone marrow ≥5% 4. Basophils in peripheral blood ≥7% 5. Basophils in bone marrow ≥3% 6. Platelet count ≥700 x109/L 7. Splenomegaly ≥10 cm below costal margin Accelerated phase: a) Blasts in peripheral blood ≥ 15% b) Basophils in peripheral blood ≥ 20% c) Blasts and promyelocytes in peripheral blood ≥30% d) Platelet count ≤100x109/L e) Cytogenetic clonal development |
Stage I: 0–1 factor Stage II: 2 factors Stage III: 3 or more factors Stage IV: accelerated phase |
a) 406 cases b) Chemotherapy |
Median OS Stage I-56 months Stage II-45 months Stage III-30 months Stage IV-30 months |
Kantarjian et al (1985) [21] | 1. Circulating basophils 2. Basophils in bone marrow 3. Race 4. Age 5. Additional chromosome abnormalities |
Low risk- HR < 0.8 Intermediate risk- HR 0.8 to 1.39 High risk- HR > 1.39 |
a) 303 cases b) Busulphan or hydroxyurea or OAP (vincristine, cytarabine, prednisolone) + anthracycline or cyclophosphamide or splenomegaly |
Median OS: Low risk- 53 months Intermediate risk- 39 months High risk- 25 months |
Sokal score (1984) [5] | 1. Age 2. Spleen size below costal margin (cm) 3. Platelet count 4. Blasts in peripheral blood (%) |
Low risk: < 0.8 Intermediate risk: 0.8–1.2 High risk: > 1.2 |
a) 813 cases, Europe, USA b) Busulfan or hydroxyurea |
OS at two years: Low risk- 90% High risk- 65% |
Hasford score (1996) [22] | 1. Age 2. Spleen size below costal margin (cm) 3. Erythroblasts in peripheral blood (%) 4. Eosinophils in peripheral blood (%) 5. Gender |
Low risk: < 1.4 Intermediate risk: 1.4–2.0 High risk: > 2.0 |
a) 490 cases, Germany b) Busulphan, hydroxyurea, IFNa |
Five years OS: Low risk- 90% |
Euro score (1998) [6] | 1. Age 2. Spleen size below costal margin (cm) 3. Blasts in peripheral blood (%) 4. Eosinophils in peripheral blood (%) 5. Basophils in peripheral blood (%) 6. Platelet count |
Low risk: ≤ 780 Intermediate risk: > 780 ≤ 1480 High risk: > 1480 |
a) 1303 cases, Europe, Japan, USA b) IFNa |
Median OS: Low risk- 98 months Intermediate risk- 65 months High risk-42 months |
EUTOS score (2011) [7] | 1. Basophils in peripheral blood (%) 2. Spleen size below costal margin (cm) |
Low risk: ≤ 87 High risk: > 87 |
a) 2060 cases,Europe b) TKI (imatinib 400mg/d in 41% cases, imatinib 400mg/d +LDAC or IFNa in 34% cases, imatinib 600–800mg/d in 25% cases |
CCyR at 18 months: Low risk-86% High risk-66% PFS at five years: Low risk- 90% High risk- 82% |
IFNa- interferon alpha, LDAC- low dose cytosine arabinoside