Table 1.
Clinical and laboratory features in 25 patients with World Health Organization defined atypical chronic myeloid leukemia (aCML), stratified by the presence or absence of TET2 mutations.
| Variable | All patients with aCML (n= 25) | TET2 mutated aCML patients (n=4) | TET2 wild type aCML patients (n=21) | P value |
|---|---|---|---|---|
|
| ||||
| Age in years; median (range) | 70 (49–91) | 68 (64–76) | 71 (49–91) | 0.5 |
|
| ||||
| Males; n (%) | 21 (84%) | 4 (100%) | 17 (81%) | 0.3 |
|
| ||||
| Hemoglobin g/dL; median (range) | 9.1 (6.3–14.9) | 9.1 (8.7–12.3) | 9.7 (6.3–14.9) | 0.6 |
|
| ||||
| MCV femtoliter; median (range) | 96 (82–111) | 92 (86–103) | 97 (82–111) | 0.7 |
|
| ||||
| WBC × 109/L; median (range) | 32 (8.3–192.7) | 27.2 (14.4–21.8) | 34.6 (8.3–192.7) | 0.7 |
|
| ||||
| ANC × 109/L; median (range) | 20.4 (0.4–153.2) | 19.7 (12–77.9) | 20.7 (0.4–153.2) | 0.9 |
|
| ||||
| AMC × 109/L; median (range) | 1.0 (0–5.4) | 1.6 (0.1–4.8) | 1.0 (0–5.4) | 0.9 |
|
| ||||
| ALC × 109/L; median (range) | 2.1 (0–7.7) | 3.6 (0.8–6.1) | 2.1 (0–7.7) | 0.6 |
|
| ||||
| ABC × 109/L; median (range) | 0.5 (0–2.2) | 0.1 (0–1.2) | 0.5 (0–2.2) | 0.4 |
|
| ||||
| AEC × 109/L; median (range) | 0.6 (0–5.7) | 0.6 (0–1.2) | 0.6 (0–5.7) | 0.7 |
|
| ||||
| Platelets × 109/L; median (range) | 95 (12–647) | 120 (95–398) | 81 (12–647) | 0.2 |
|
| ||||
| Presence of circulating immature myeloid cells; n (%) | 25 (100%) | 4 (100%) | 21 (100%) | 0.9 |
|
| ||||
| PB blast %; median (range) | 1 (0–12) | 1 (1–2) | 1 (0–12) | 0.9 |
|
| ||||
| BM blast % ; median (range) | 2 (0–15) | 1.5 (0–4) | 3 (0–15) | 0.4 |
|
| ||||
| BM cellularity % | 95 (80–100) | 92 (90–100) | 95 (80–100) | 0.7 |
|
| ||||
| BM dyserythropoiesis; yes (%) | 4 (16%) | 1 (25%) | 3 (14%) | 0.6 |
|
| ||||
| BM dysmegakaryocytopoiesis; yes (%) | 5 (20%) | 1 (25 %) | 4 (19%) | 0.8 |
|
| ||||
| Red blood cell transfusion dependence | 16 (64%) | 3 (75%) | 13 (62%) | 0.6 |
|
| ||||
| Palpable splenomegaly; n (%) | 13 (52%) | 2 (50%) | 11 (52%) | 0.9 |
| Palpable splenomegaly>10 cm below LCM; n (%) | 8 (32%) | 2 (50%) | 6 (29%) | 0.4 |
|
| ||||
| *Cytogenetic abnormalities; n (%) | ||||
| Normal | 14 (67%) | 2 (50%) | 12 (57%) | |
| Trisomy 8 | 6 (27%) | 2 (50%) | 4 (21%) | 0.6 |
| Trisomy 9 | 1 (4%) | 0 (0%) | 1 (5%) | |
| Trisomy 21 | 1 (4%) | 0 (0%) | 1 (5%) | |
|
| ||||
| Leukemic transformations; n (%) | 2 (8%) | 0 (0%) | 2 (10%) | 0.5 |
|
| ||||
| Deaths; n (%) | 17 (68%) | 3 (75%) | 14 (67%) | 0.7 |
|
| ||||
| Median follow up in months (range) | 11 (1–29) | 5 (1–6) | 13 (1–29) | 0.03 |
Key: aCML- Atypical Chronic Myeloid Leukemia, MCV- mean corpuscular volume, WBC- white blood cell count; ANC- absolute neutrophil count; AMC- absolute monocyte count; ALC- absolute lymphocyte count; ABC-absolute basophil count; AEC-absolute eosinophil count; PB- peripheral blood; BM- bone marrow; LCM- lower costal margin.
Cytogenetic studies were available for 23 patients with atypical chronic myeloid leukemia at diagnosis. In this study loss of chromosome Y was considered normal. One patient showed the following cytogenetic abnormality – 46,XY, inv(6)(p11.2q25)?c[30]- that was considered constitutional.