Clinical and hematological characteristics of JMML genetic subtypes at diagnosis
| . | . | CBL (N = 43) . | NF1 (N = 51) . | PTPN11 (N = 180) . | KRAS (N = 68) . | NRAS (N = 65) . | P . |
|---|---|---|---|---|---|---|---|
| Clinical | Age (y), median (range) | 0.9 (0.1‐5.0) | 2.8 (0.3‐12.3) | 2.1 (0.1‐16.3) | 0.9 (0.1‐10.0) | 1.2 (0.1‐13.2) | <.01 |
| Male sex, % | 56 | 65 | 60 | 75 | 74 | .07 | |
| Spleen (palpable cm ↓ CM), median (range)* | 5 (0‐15) | 4 (0‐15) | 4 (0‐13) | 5 (0‐15) | 5 (0‐15) | NS | |
| PB | WBC (×109/L), median (range) | 27 (8‐113) | 32 (3‐131) | 30 (2‐424) | 21 (4‐177) | 41 (5‐312) | <.01 |
| Blasts (%), median (range) | 0 (0‐9) | 2 (0‐25) | 2 (0‐21) | 2 (0‐21) | 2 (0‐18) | <.01 | |
| Precursors (%), median (range)† | 4 (0‐42) | 5 (0‐31) | 9.5 (0‐56) | 7 (0‐65) | 8.2 (0‐45) | .05 | |
| Eosinophils (%), median (range) | 2 (0‐8) | 2 (0‐15) | 1 (0‐26) | 2 (0‐9) | 2 (0‐18) | .01 | |
| AMC (×109/L), median (range) | 4.0 (1‐48) | 7.6 (1‐26) | 4.1 (0‐237) | 5.6 (0‐40) | 6.2 (9‐232) | <.01 | |
| Platelet count (×106/L), median (range) | 92 (14‐289) | 129 (15‐357) | 50 (5‐373) | 42 (10‐182) | 60 (9‐232) | <.01 | |
| Hemoglobin (g/100 mL), median (range) | 9.0 (5‐13) | 10.0 (4‐13) | 9.1 (3‐13) | 8.7 (4‐14) | 8.9 (4‐13) | NS | |
| HbF (%), median (range) | 4 (0‐59) | 23 (1‐75) | 18 (0‐100) | 10 (0‐94) | 13 (0‐70) | <.01 | |
| BM | MP:EP, median (range) | 5.4 (1.1‐45) | 5.7 (0.4‐23) | 3.7 (0.1‐233) | 2.9 (0.3‐219) | 3.5 (0.5‐97) | <.01 |
| Blasts (%), median (range) | 3 (0‐20) | 5 (0‐27) | 4 (0‐34) | 4 (0‐20) | 4 (0‐20) | NS | |
| Eosinophils (%), median (range) | 2 (0‐15) | 3 (0‐12) | 2 (0‐33) | 3 (0‐18) | 2 (0‐18) | NS | |
| Karyotype | Normal (%) | 97 | 77 | 69 | 40 | 86 | <.01 |
| Monosomy 7 (%) | 0 | 13 | 21 | 52 | 7 | ||
| Other aberration (%) | 3 | 10 | 10 | 8 | 7 |
| . | . | CBL (N = 43) . | NF1 (N = 51) . | PTPN11 (N = 180) . | KRAS (N = 68) . | NRAS (N = 65) . | P . |
|---|---|---|---|---|---|---|---|
| Clinical | Age (y), median (range) | 0.9 (0.1‐5.0) | 2.8 (0.3‐12.3) | 2.1 (0.1‐16.3) | 0.9 (0.1‐10.0) | 1.2 (0.1‐13.2) | <.01 |
| Male sex, % | 56 | 65 | 60 | 75 | 74 | .07 | |
| Spleen (palpable cm ↓ CM), median (range)* | 5 (0‐15) | 4 (0‐15) | 4 (0‐13) | 5 (0‐15) | 5 (0‐15) | NS | |
| PB | WBC (×109/L), median (range) | 27 (8‐113) | 32 (3‐131) | 30 (2‐424) | 21 (4‐177) | 41 (5‐312) | <.01 |
| Blasts (%), median (range) | 0 (0‐9) | 2 (0‐25) | 2 (0‐21) | 2 (0‐21) | 2 (0‐18) | <.01 | |
| Precursors (%), median (range)† | 4 (0‐42) | 5 (0‐31) | 9.5 (0‐56) | 7 (0‐65) | 8.2 (0‐45) | .05 | |
| Eosinophils (%), median (range) | 2 (0‐8) | 2 (0‐15) | 1 (0‐26) | 2 (0‐9) | 2 (0‐18) | .01 | |
| AMC (×109/L), median (range) | 4.0 (1‐48) | 7.6 (1‐26) | 4.1 (0‐237) | 5.6 (0‐40) | 6.2 (9‐232) | <.01 | |
| Platelet count (×106/L), median (range) | 92 (14‐289) | 129 (15‐357) | 50 (5‐373) | 42 (10‐182) | 60 (9‐232) | <.01 | |
| Hemoglobin (g/100 mL), median (range) | 9.0 (5‐13) | 10.0 (4‐13) | 9.1 (3‐13) | 8.7 (4‐14) | 8.9 (4‐13) | NS | |
| HbF (%), median (range) | 4 (0‐59) | 23 (1‐75) | 18 (0‐100) | 10 (0‐94) | 13 (0‐70) | <.01 | |
| BM | MP:EP, median (range) | 5.4 (1.1‐45) | 5.7 (0.4‐23) | 3.7 (0.1‐233) | 2.9 (0.3‐219) | 3.5 (0.5‐97) | <.01 |
| Blasts (%), median (range) | 3 (0‐20) | 5 (0‐27) | 4 (0‐34) | 4 (0‐20) | 4 (0‐20) | NS | |
| Eosinophils (%), median (range) | 2 (0‐15) | 3 (0‐12) | 2 (0‐33) | 3 (0‐18) | 2 (0‐18) | NS | |
| Karyotype | Normal (%) | 97 | 77 | 69 | 40 | 86 | <.01 |
| Monosomy 7 (%) | 0 | 13 | 21 | 52 | 7 | ||
| Other aberration (%) | 3 | 10 | 10 | 8 | 7 |
Analysis of 407 children diagnosed between 1990 and 2018 registered to the European Working Group of MDS in Childhood (EWOG‐MDS).
AMC, absolute monocyte count; CM, costal margin; EP, erythropoiesis; MP, myelopoiesis.
Large spleens in pelvis set as 15 cm below costal margin.
Promyelocytes + myelocytes + metamyelocytes + normoblasts.