Table 3.
Prognostic scoring systems for chronic myelomonocytic leukemia
| Prognostic Score | Year | Number of Patients | External Validation | Variables included in the final model | Median Survival in months | Transformation into AML | |||
|---|---|---|---|---|---|---|---|---|---|
| Low risk | Intermediate-1 risk | Intermediate-2 risk | High risk | ||||||
| Onida et al5 (MDAPS) | 2002 | 213 | No | 1. Hemoglobin <12 gm/dL 2. Circulating immature myeloid cells 3. Absolute lymphocyte count >2.5 x 109/L 4. Bone marrow blasts >10% |
24 | 15 | 8 | 5 | 19% developed AML after a median time of 7 months |
| Germing et al4 (Dusseldorf score for CMML) | 2004 | 288 | No | 1. Bone marrow blasts ≥5% 2. LDH >200 U/L 3. Hemoglobin ≤9 gm/dl 4. Platelets ≤100 x 109/L |
93 | 26 | 11 | 8%, 23% and 23% at 5 years, respectively | |
| Such et al42 (CPSS Model) | 2013 | 578 | Yes, in 274 patients | 1. CMML FAB type 2. CMML WHO type 3. CMML-specific cytogenetics 4. RBC transfusion dependence |
72 | 31 | 13 | 5 | Probability of AML evolution at 5 years, 13%, 29%, 60%, and 73%, respectively |
| Itzykson et al22 (GFM Model) | 2013 | 312 | Yes, 165 patients | 1. Age >65 years 2. WBC >15x109/L 3. Anemia 4. Platelets <100 x109/L 5. ASXL1 mutation |
Not reached | 38.5 | 14.4 | AML-free survival was 56.0, 27.4, and 9.2 months, respectively | |
| Patnaik et al34 (Mayo Model) | 2013 | 226 | Yes, 268 patients | 1. Increased absolute monocyte count >10×109/L 2. Presence of circulating blasts 3. Hemoglobin <10 gm/dL 4. Platelet count <100 ×109/L |
32 | 18.5 | 10 | NR | |
| Patnaik et al (Mayo Molecular Model) | 2014 | 466 | No | 1. Increased absolute monocyte count >10×109/L 2. Presence of circulating blasts 3. Hemoglobin <10 gm/dL 4. Platelet count <100 ×109/L 5. Frameshift and nonsense ASXL1 mutations |
97 | 59 | 31 | 16 | At a median follow up of 23 months, 75 (16%) leukemic transformations occurred. |
| Elena et al (CPSS-Mol) | 2016 | 214 | 260 | 1. Genetic risk groups as defined by *CPSS cytogenetic risk stratification and gene mutations involving ASXL1, NRAS, SETBP1 and RUNX1. 2. Bone marrow blasts ≥ 5%. 3. WBC count ≥ 13x109/L 4. Red blood cell transfusion dependancy |
Not reached | 64 | 37 | 18 | 48 months cumulative incidence of AML evolution; 0%, 3%, 21% and 48%, respectively. |
Key: MDAPS- MD Anderson Prognostic Scoring System, CPSS- CMML specific prognostic scoring system, CMML- chronic myelomonocytic leukemia, GFM- Groupe Francophone des Myélodysplasies, LDH- lactate dehydrogenase, FAB- French American British, WHO – World Health Organization, WBC- white blood cell count.
The CPSS-Mol used a genetic risk group stratification that assigned a score of 0 for low risk cytogenetics and absence of ASXL1/NRAS/SETBP1/RUNX1 mutations, a score of 1 for intermediate risk cytogenetics and mutations involving ASXL1/SETBP1 and NRAS, and a score of 2 for high risk cytogenetics and RUNX1 mutations