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To share and enhance best practice management of CML, experts and interested clinicians can discuss difficult or interesting CML patient cases here. Clinicians submit a brief history of the patient and the case for discussion (no more than 200 words) by posting it in this forum ("+ NEW TOPIC" button below).

Each clinical case will be forwarded to the iCMLf expert clinical panel for a brief independent response. Consideration should be given to patient confidentiality. Details that are not critical to the case can be changed to preserve anonymity.

As a full clinical history is necessary for accurate comment, cases and comments on the Forum are only accepted from clinicians. If individual patients have a specific question we encourage them to contact their healthcare provider.

DISCLAIMER: The iCMLf does not recommend or endorse any specific tests, physicians, products, procedures, or opinions, and disclaims any representation, warranty, or guaranty as to the same. Reliance on any information provided in this Forum is solely at your own risk.

TOPIC: CML congenital cardiac lesions

CML congenital cardiac lesions 5 months 2 days ago #1609

This is a 37-y-o patient with recently diagnosed CML EUTOS score low. She has a history of congental cyanotic cardiopathy that has been surgically corrected on 2 occasions. She has a complete AV block and is on furosemide 20mg/d, ramipril 5md/d and carbamazepine 400mg/d (this is due na epitlogenic focus after a TIA), and leads a normal life.
what is the best TKI to start treatment with? Bosutinib?

CML congenital cardiac lesions 4 months 4 weeks ago #1610

  • Jeff Lipton
  • Jeff Lipton's Avatar
It is a good question, but some more information would be helpful. Does she have a pacemaker? Any history of pleural effusions post operatively? Any history of heart failure at this time? How does it manifest?
If there is no history of peripheral edema, my choice for first line therapy would actually be imatinib. Pleural inflammation in the past with surgery might put her at risk with Dasatinib. Her hypertension might get worse with Bosutinib. Since this is not vascular disease related cardiac problems, and if she otherwise has no other risk factors and she has a pacemaker, nilotinib would probably be my second choice after imatinib.


ORIGINAL CASE:
This is a 37-y-o patient with recently diagnosed CML EUTOS score low. She has a history of congental cyanotic cardiopathy that has been surgically corrected on 2 occasions. She has a complete AV block and is on furosemide 20mg/d, ramipril 5md/d and carbamazepine 400mg/d (this is due na epitlogenic focus after a TIA), and leads a normal life.
what is the best TKI to start treatment with? Bosutinib?

CML congenital cardiac lesions 4 months 4 weeks ago #1611

  • Andrija Bogdanovic
  • Andrija Bogdanovic's Avatar
hello, I would be quite conservative in this lady with congenital heart disease after double life saving surgery and AV block. I would consider slower move towards CML control, therefore even in case of some manifestations of her heart disease (peripheral edema, but she is on furosemide) I would start with imatinib standard dose especially EUTOS is low. It would help to have ELTS survival estimate as one of possible leads in choice, but dasatinib is not suitable due to open heart surgery and possible effusions, and similarly nilotinib with metabolic effects are not my choice in the moment. Both, dasa and nilo may have QT prolongation and in such a case many unusual things could happen by Marphy. It would be a problem if she needs second line treatment? Moreover I have checked with such treatment there would no significant interactions with TKI's at all.

ORIGINAL CASE:
This is a 37-y-o patient with recently diagnosed CML EUTOS score low. She has a history of congental cyanotic cardiopathy that has been surgically corrected on 2 occasions. She has a complete AV block and is on furosemide 20mg/d, ramipril 5md/d and carbamazepine 400mg/d (this is due na epitlogenic focus after a TIA), and leads a normal life.
what is the best TKI to start treatment with? Bosutinib?
Last Edit: 4 months 4 weeks ago by Nicola.

CML congenital cardiac lesions 4 months 4 weeks ago #1612

  • manuel abecasis
  • manuel abecasis's Avatar
Dear Jeff,
Thank you for your comments.
She does have a pace-maker and had a history of heart failure NYHA class IV but no pleural effusions. I have reviewed the cardiac and vascular toxicities of both bosutinib and imatinib and I will go for Imatinib as you suggest.


ORIGINAL CASE:
This is a 37-y-o patient with recently diagnosed CML EUTOS score low. She has a history of congental cyanotic cardiopathy that has been surgically corrected on 2 occasions. She has a complete AV block and is on furosemide 20mg/d, ramipril 5md/d and carbamazepine 400mg/d (this is due na epitlogenic focus after a TIA), and leads a normal life.
what is the best TKI to start treatment with? Bosutinib?
Last Edit: 4 months 4 weeks ago by Nicola.

CML congenital cardiac lesions 4 months 4 weeks ago #1613

  • manuel abecasis
  • manuel abecasis's Avatar
Dear Dr Bogdanovic
Thank you for your comments.
She has a ELTS score of 0,6 that is low risk. I agree with you Imatinib is probably the TKI to start with.
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