Criteria for chronic, accelerated, and blast phases of CML7
| CML-CP . | CML-AP . | CML-BC . |
|---|---|---|
| Must meet all of the following criteria: | Must meet 1 or more of the following criteria: | Must meet 1 or more of the following criteria: |
| Documentation of t(9;22) or the Bcr-Abl fusion gene | Blasts 10%-19% of peripheral blood white cells or bone marrow cells | Blasts ≥ 20% of peripheral blood white cells or bone marrow cells |
| Bone marrow blasts < 10% | Peripheral blood basophils at least 20% | Extramedullary blast proliferation |
| Does not meet any criteria for accelerated phase or blast crisis | Persistent thrombocytopenia (100 × 109/L) unrelated to therapy, or persistent thrombocytosis (1000 × 109/L) unresponsive to therapy | Large foci or clusters of blasts in bone marrow biopsy |
| Increasing spleen size and increasing WBC count unresponsive to therapy | ||
| Cytogenetic evidence of clonal evolution (ie, the appearance of an additional genetic abnormality that was not present in the initial specimen at the time of diagnosis of chronic phase CML) | ||
| Megakaryocytic proliferation in sizable sheets and clusters, associated with marked reticulin or collagen fibrosis, and/or severe granulocytic dysplasia, should be considered as suggestive of CML-AP; these findings have not yet been analyzed in large clinical studies; however, so it is not clear whether they are independent criteria for accelerated phase; they often occur simultaneously with one or more of the other features listed |
| CML-CP . | CML-AP . | CML-BC . |
|---|---|---|
| Must meet all of the following criteria: | Must meet 1 or more of the following criteria: | Must meet 1 or more of the following criteria: |
| Documentation of t(9;22) or the Bcr-Abl fusion gene | Blasts 10%-19% of peripheral blood white cells or bone marrow cells | Blasts ≥ 20% of peripheral blood white cells or bone marrow cells |
| Bone marrow blasts < 10% | Peripheral blood basophils at least 20% | Extramedullary blast proliferation |
| Does not meet any criteria for accelerated phase or blast crisis | Persistent thrombocytopenia (100 × 109/L) unrelated to therapy, or persistent thrombocytosis (1000 × 109/L) unresponsive to therapy | Large foci or clusters of blasts in bone marrow biopsy |
| Increasing spleen size and increasing WBC count unresponsive to therapy | ||
| Cytogenetic evidence of clonal evolution (ie, the appearance of an additional genetic abnormality that was not present in the initial specimen at the time of diagnosis of chronic phase CML) | ||
| Megakaryocytic proliferation in sizable sheets and clusters, associated with marked reticulin or collagen fibrosis, and/or severe granulocytic dysplasia, should be considered as suggestive of CML-AP; these findings have not yet been analyzed in large clinical studies; however, so it is not clear whether they are independent criteria for accelerated phase; they often occur simultaneously with one or more of the other features listed |
Like adults, most children will present in chronic phase.