I. Classic MPD | 1. BCR-ABL-positive
2. BCR-ABL-negative | Chronic myeloid leukemia (CML)Polycythemia vera (~100% JAK2V617F+) Essential thrombocythemia (~50% JAK2V617F+) Myelofibrosis (~50% JAK2V617F+) |
II. Atypical MPD | 1. Chronic myelomonocytic leukemia | |
| 2. Juvenile myelomonocytic leukemia (frequent PTP11, NF1, and RAS mutations) | |
| 3. Chronic neutrophilic leukemia (~20% JAK2V617F+) | |
| 4. Chronic eosinophilic leukemia/eosinophilic MPD | PDGFRA-rearranged (e.g., FIP1L1-PDGFRA) PDGFRB-rearranged (e.g., TEL/ETV6-PDGFRB) FGFR1-rearranged (e.g., ZNF198/FIM/RAMP-FGFR1) (a.k.a. 8p11 myeloproliferative syndrome) Molecularly undefined |
| 5. Hypereosinophilia syndrome | |
| 6. Chronic basophilic leukemia | |
| 7. Systemic mastocytosis | PDGFRA-rearranged (e.g., FIP1L1-PDGFRA) KIT-mutated (e.g., KITD816V) Molecularly undefined |
| 8. Unclassified MPD (~20% JAK2V617F+) | |