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Patient with an extramedullary blast phase

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International CML Foundation
Topic Author

10 years 10 months ago #382 by International CML Foundation

Patient with an extramedullary blast phase was created by International CML Foundation

37 year old female patients with history of CML. She received imatinib at diagnosis and did not achieve a complete cytogenetic response. There were some issues with compliance. Eventually she was changed to receive nilotinib (no mutations were identified by direct sequencing). There was a transient cytogenetic response but eventually the patient progressed to blast phase. She received ponatinib, achieved a CHR and minor cytogenetic response and received and allogeneic stem cell transplant from an unrelated donor. There are no transplant-related complications but the PCR has remained positive after SCT thus ponatinib was re-started at a dose of 15 mg daily. There has been mild thrombocytopenia (platelets around 60 x109/L) and the PCR became negative. Now presents with an isolated skin lesion in the back that was biopsied and reported as granulocytic sarcoma (FISH in the biopsy is positive for bcr-abl). The bone marrow and peripheral blood still show no evidence of disease by morphology, cytogenetics or PCR. What would be the approach for this patient with an extramedullary blast phase?

Jorge Cortes
Topic Author

10 years 10 months ago #383 by Jorge Cortes

Replied by Jorge Cortes on topic Re: Patient with an extramedullary blast phase

This is a complicated case. The presence of extramedullary disease establishes the diagnosis of blast phase. This patient thus needs a different therapy. I would consider using AML-like chemotherapy in combination with a TKI. For the chemotherapy, we have used low-dose ara-C-based combination with low-dose idarubicin in some patients with good success. Although if patient is in good overall condition, perhaps a higher-dose ara-C regimen is a better option. For the TKI I would consider bosutinib or dasatinib at this time. It is also important to consider DLI or a second transplant. Unfortunately these instances of isolated extramedullary disease can be difficult to treat and you frequently end up chasing the tumors. Local radiotherapy may help eliminate the current lesion, but is not enough for long-term control of the disease.

Pankaj Malhotra
Topic Author

10 years 10 months ago #384 by Pankaj Malhotra

Replied by Pankaj Malhotra on topic Re: Patient with an extramedullary blast phase

Would it be helpful to further characterize the granulocytic sarcome in this case whether the predominant cells are blast cells or fully differentiated granulocytic cells. If predominant cells are blast cells then treatment line would be on the lines of high dose chemotherapy and second transplant. If predominant cells are of immature granulocytic series without many blasts, would then case be made to treat this with local radiation only?

John Goldman
Topic Author

10 years 10 months ago #385 by John Goldman

Replied by John Goldman on topic Re: Patient with an extramedullary blast phase

nghI tend to agree largely with Jorge Cortes. Despite the terminology 'granulocytic sarcoma' it might be useful to exclude the possibility that this was in fact a predominantly lymphoid blastic event. Probably not. I would certainly recommend local radiotherpy if this can be administered without damaging surrounding tissues. Nonetheless it is likely that the blastic style disease has disseminated widely. I would therefore continue TKI, probably best dasatinib, and add chemotherapy very cautiously because I would not expect a post allograft patient to tolerate full doses. DLI is not notoriously effective in advanced phase CML but it could be considered if the donor is available.

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