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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: -7 at diagnosis

-7 at diagnosis 2 months 1 week ago #1367

Hello! I want to know what do you think about this patient. He is a 22 years old male. Diagnosis CML on March 17. High Sokal. Chronic phase. He received Hydroxiurea and then Imatinib, 400 mg started on April 20. Pacient in Complete Hematologic Response before a month of treatment. I received cytogenetic inform: 11 metaphases, 46,XY,-7, t(9;22)(q34;q11.2), +12.
Which is the best treatment for this patient? Is the patient in Chronic Phase or Accelereted Phase?
Thank you!

-7 at diagnosis 2 months 4 days ago #1369

  • Andrija Bogdanovic
  • Andrija Bogdanovic's Avatar
This young guy may be in chronic phase CML due to blast count in the bone marrow, without typical cytogenetic aberrations seen in accelerated or blastic phase. I am wondering what are the other scores, especially EUTOS and ELTS (you have it on ELN webpage), and what is the main factor responsible for high Sokal (spleen, blasts, baso?). ELTS may help in estimation of risk of dying from CML even it not includes genetics. Unfortunately, monosomy 7 is quite strong adverse chromosomal change in hematology, often associated with myelotoxic effects like chemicals, irradiation etc, and therefore it raises many concerns in this case. With only trisomy 12 I would be more reluctant but with mono7, this guy is really at risk for further failure and progression.
The best treatment is to use the strongest TKI you have in local setting to suppress this Ph+ clone and to follow up strictly to ELN guidelines, with full karyotype and PCR at 3 and 6 months. Moreover, if his health insurance covers, I will start search for allogeneic donor (sibling and unrelated), because we can expect different scenarios.
In case you have possibility to perform mutation, do it, just to have information weather T315I is present or not, but it is not obligatory. Concerning TKI choice, it depends on resources. As you have started with imatinib and achieved CHR within one month of treatment, I probably would continue that up to 3 months evaluation. Probably, in case of having dasatinib, I would prefer that drug as choice in the first line setting. Nilotinib might be also OK. You have started imatinib 400, but in this case if only imatinib treatment available, I would probably use Imatinib 800 due to those adverse karyotype. Nevertheless, as you have CHR continue imatinib 400 with evaluation at 3 months (bone marrow blast count, karyotype, PCR) and in any not really optimal response, escalate imatinib to 800 or switch to another drug if available. I expect suboptimal response at best, and anything better is good for patient. In case of cytopenia and need for drug holiday I will put more efforts towards allogeneic transplant. I will not use any growth factor for shortening of neutropenia in such a case.
I am looking forward to have further follow up information.
Dr Andrija Bogdanovic, Belgrade, Serbia

-7 at diagnosis 2 months 3 days ago #1370

The patient has in PB 4% baso, 4% blasts, and 4% Promyelocytes. Spleen from costmargin 20 cm.
BCR-ABL/ABL at dagnosis 23,3%.
He has a sister, I will do the HLA study.
I have started Imatinib before the cytogenetic inform. I will do PCR and karytype at 3 and 6 months.

Additional information: Patient has ELTS Score intermediate risk, and EUTOS high risk.
BM 2% blasts, and 2% promyelocitos.

Thank you for your reply.

Original case below:
Hello! I want to know what do you think about this patient. He is a 22 years old male. Diagnosis CML on March 17. High Sokal. Chronic phase. He received Hydroxiurea and then Imatinib, 400 mg started on April 20. Pacient in Complete Hematologic Response before a month of treatment. I received cytogenetic inform: 11 metaphases, 46,XY,-7, t(9;22)(q34;q11.2), +12.
Which is the best treatment for this patient? Is the patient in Chronic Phase or Accelereted Phase?
Thank you!
Last Edit: 2 months 3 days ago by Melissa Davis-Bishop.

-7 at diagnosis 2 months 3 days ago #1371

  • jeff lipton
  • jeff lipton's Avatar
I think Andrija has hit the nail right on the head. Although morphologically, he is in CP, the adverse chromosomes are very worrisome. German and Italian studies would indicate a worse response and response duration because of the additional chromosome abnormalities. The monosomy 7 is especially worrisome as there may be a risk here of MDS/AML as well. It is not clear whether all the abnormalities listed here are in the same clone or multiple clones, but I think this matters little. I believe that allografting should be considered early here. If a donor is readily available and the young man can be taken to transplant soon, then probably no need to switch to a 2G TKI. If there is a delay or no donor is available, then I agree completely that dasatinib or nilotinib would be preferred. I do not think that the 3-month result should decide on allografting. I would proceed under all conditions if possible.
Moderators: Melissa Davis-Bishop
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