Clinical Case Discussion Forum
To share and enhance best practice management of CML, experts and interested clinicians can discuss difficult or interesting CML cases here. Physicians submit a brief history of the patient and the case for discussion using this forum.
Blast crisis with immature double population
Male patient, 42 years old, diagnosed with CML (positive BCR / ABL), in the diagnosis presented in bone marrow immunophenotyping two populations of blasts: t lymphoid populations (0.45%) + myeloblasts (1.34%) totaling 1.78 immature cells). Description of grade 3 fibrosis. Treatment initiated with imatinib.
It evolved with the appearance of multiple tumors and showed granulocytic sarcoma.
Questions :
In treatment, ITK Dasatinib should be associated with which systemic chemotherapy. Should I opt for chemotherapy directed at the myeloid component (7 + 3) or T lymphoid (HyperCVAD) or both (FLAG-IDA?
When choosing therapy, should we consider grade 3 bone marrow fibrosis?
There's a new bone marrow evaluation schedule with immunophenotyping that we still don't have the result for. In evaluating possible donors for ongoing transplants.
I would resist to apply a full dose combination chemotherapy. Recovery of normal hematopoiesis is impossible in CML and the whole treatment aims to prepare for transplant.
Therefore, Dasatinib 100 mg/d should be the start, combined with an individually dosed chemotherapy with Ara-C and a intermediate dose of an anthracyclin.
A full dosed 7+3 or similar will result in long lasting cytopenias and may be disadvantageous.
With some more information about timing (diagnosis until myeloid sarcoma, histology of the lesions (% blasts), BCR-ABL mutations, cytogenetics) I would be happy to provide more detailed advice if you would like me to.