Table 2

Criteria for CML, accelerated phase

CML, accelerated phase criteria
Any 1 or more of the following hematologic/cytogenetic criteria or response-to-TKI criteria: 
• Persistent or increasing WBC (>10 × 109/L), unresponsive to therapy “Provisional” response-to-TKI criteria 
• Persistent or increasing splenomegaly, unresponsive to therapy • Hematologic resistance to the first TKI (or failure to achieve a complete hematologic response* to the first TKI) or 
• Persistent thrombocytosis (>1000 × 109/L), unresponsive to therapy • Any hematological, cytogenetic, or molecular indications of resistance to 2 sequential TKIs or 
• Persistent thrombocytopenia (<100 × 109/L) unrelated to therapy • Occurrence of 2 or more mutations in BCR-ABL1 during TKI therapy 
• 20% or more basophils in the PB  
• 10%-19% blasts in the PB and/or BM  
• Additional clonal chromosomal abnormalities in Ph+ cells at diagnosis that include “major route” abnormalities (second Ph, trisomy 8, isochromosome 17q, trisomy 19), complex karyotype, or abnormalities of 3q26.2  
• Any new clonal chromosomal abnormality in Ph+ cells that occurs during therapy  
CML, accelerated phase criteria
Any 1 or more of the following hematologic/cytogenetic criteria or response-to-TKI criteria: 
• Persistent or increasing WBC (>10 × 109/L), unresponsive to therapy “Provisional” response-to-TKI criteria 
• Persistent or increasing splenomegaly, unresponsive to therapy • Hematologic resistance to the first TKI (or failure to achieve a complete hematologic response* to the first TKI) or 
• Persistent thrombocytosis (>1000 × 109/L), unresponsive to therapy • Any hematological, cytogenetic, or molecular indications of resistance to 2 sequential TKIs or 
• Persistent thrombocytopenia (<100 × 109/L) unrelated to therapy • Occurrence of 2 or more mutations in BCR-ABL1 during TKI therapy 
• 20% or more basophils in the PB  
• 10%-19% blasts in the PB and/or BM  
• Additional clonal chromosomal abnormalities in Ph+ cells at diagnosis that include “major route” abnormalities (second Ph, trisomy 8, isochromosome 17q, trisomy 19), complex karyotype, or abnormalities of 3q26.2  
• Any new clonal chromosomal abnormality in Ph+ cells that occurs during therapy  

Large clusters or sheets of small, abnormal megakaryocytes, associated with marked reticulin or collagen fibrosis in biopsy specimens may be considered as presumptive evidence of AP, although these findings are usually associated with 1 or more of the criteria listed above.

*

Complete hematologic response: WBC, <10 × 109/L; platelet count, <450 × 109/L, no immature granulocytes in the differential, and spleen nonpalpable.

The finding of bona fide lymphoblasts in the blood or marrow, even if <10%, should prompt concern that lymphoblastic transformation may be imminent and warrants further clinical and genetic investigation; 20% or more blasts in blood or BM, or an infiltrative proliferation of blasts in an extramedullary site is CML, blast phase.

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