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Appendix 2 WHO classifi cation of tumours of the haematopoietic and lymphoid tissues / 427
Myeloproliferative n eoplasms Myelodysplastic s yndromes
Chronic myelogenous leukaemia, BCR – ABL1 Refractory cytopenia with unilineage dysplasia
positive Refractory anaemia
Chronic neutrophilic leukaemia Refractory neutropenia
Polycythaemia vera Refractory thrombocytopenia
Primary myelofi brosis Refractory anaemia with ring sideroblasts
Essential thrombocythaemia Refractory cytopenia with multilineage dysplasia
Chronic eosinophilic leukaemia, not otherwise Refractory anaemia with excess blasts
specifi ed Myelodysplastic syndromes associated with
Mastocytosis isolated del(5q)
Cutaneous mastocytosis Myelodysplastic syndromes, unclassifi able
Systemic mastocytosis Myelodysplastic syndromes in children
Mast cell leukaemia
Mast cell sarcoma
Extracutaneous mastocytoma Acute m yeloid l eukaemia
Myeloproliferative neoplasms, unclassifi able
Acute myeloid leukaemia (AML) with recurrent
Myeloid and lymphoid neoplasms associated with
genetic abnormalities
eosinophilia and abnormalities of PDGFRA ,
AML with t(8; 21)(q22; q22),
PDGFRB or FGFR1
RUNX1 – RUNX1T1
Myeloid and lymphoid neoplasms associated
AML with inv(16)(p13.1q22) or t(16; 16)
with PDGFRA rearrangement
(p13.1; q22), CBFB – MYH11
Myeloid neoplasms associated with PDGFRB
Acute promyelocytic leukaemia with t(15; 17)
rearrangement
(q22; q11 – 12), PML – RARA
Myeloid and lymphoid neoplasms with FGFR1
AML with t(9; 11)(p22; q23), MLLT3 – MLL
abnormalities
AML with t(6; 9)(p23; q34), DEK – NUP214
AML with inv(3)(q21q26.2) or t(3; 3)(q21;
q26.2), RPN1 – EVI1
Myelodysplastic/ AML (megakaryoblastic) with t(1; 22)(p13;
m yeloproliferative n eoplasms q13), RBM15 – MKL1
AML with mutated NPM1 *
Chronic myelomonocytic leukaemia AML with mutated CEBPA *
Atypical chronic myeloid leukaemia, BCR – ABL1 AML with myelodysplasia - related changes
negative Therapy - related myeloid neoplasms
Juvenile myelomonocytic leukaemia AML, not otherwise categorized
Myelodysplastic/myeloproliferative neoplasms, AML with minimal diff erentiation
unclassifi able AML without maturation
Refractory anaemia with ringed sideroblasts AML with maturation
(RARS) associated with marked Acute myelomonocytic leukaemia
thrombocytosis * Acute monoblastic and monocytic leukaemia
Acute erythroid leukaemia
Acute erythroid leukaemia, erythroid/myeloid
* These represent provisional entities or provisional subtypes of Acute pure erythroid leukaemia
other neoplasms. They are provisional because there are insuffi - Acute megakaryoblastic leukaemia
cient data to support their being a definite entity, signifi cant
controversies about their defining features and/or uncertainty Acute basophilic leukaemia
about whether they are unique or closely related to other defi nite Acute panmyelosis with myelofi brosis
entities. Myeloid sarcoma