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Chronic myeloid
leukemia

July 25, 2023 — published by Pyramidale Communication

At Pyramidale Communication Agency, we leverage our expertise to address numerous chronic diseases. One of these is Chronic Myeloid Leukemia (CML).

Key numbers for CML

CML is the most common myeloproliferative syndrome, accounting for 15 to 20% of all leukemia cases. ^1,3
In France, approximately 700 new cases are diagnosed each year. ²
The median age of onset is 62 years for men and 64 years for women. ¹
CML is a myeloid hematologic disorder with an overall favorable prognosis with a 1-year survival rate of 96% and a 5-year survival rate of 85%. ⁴
Mortality is higher in people aged 70 or older than in younger patients. ⁴

What is CML?

Chronic myeloid leukemia is a malignant blood disorder defined by the presence of the BCR-ABL fusion gene and myeloproliferative neoplasia. ¹ It results from the t(9;22)(q34;q11) translocation, which forms the Philadelphia chromosome (Ph1). This translocation generates the BCR-ABL1chimeric oncogene (breakpoint cluster region/Abelson), coding for a fusion protein with deregulated tyrosine kinase activity. ⁵ The t(9;22) translocation is detected in 95% of CML patients. ¹

Progression of the disease

CML progresses through three phases, defined by hematological parameters: ^1,5,6

Chronic phase: Often asymptomatic. Leukemic stem cells (LSCs) remain largely confined to hematopoietic tissue. ⁵
Accelerated phase:may be suspected if the spleen enlarges again after an initial shrinkage with treatment, with worsening anemia, or with development of petechiae or ecchymoses.
Blast phase: LSCs acquire invasive capacity, allowing infiltration into organs beyond the liver and spleen, such as lymph nodes, the central nervous system, and the lungs. ⁵

Diagnostic criteria and management

The diagnosis of CML is established through a thorough medical history and physical examination a complete blood count with blood smear and bone marrow aspirate, and molecular biology tests. ^1,6

CML is most often diagnosed during the chronic phase. Investigations may be triggered by abnormal routine blood counts, or by the presence of early symptoms such as fatigue, weight loss, or abdominal pain. ^1,6 In the acute phase, patients may present with general deterioration and bone pain. In addition to splenomegaly, tumor syndrome may include lymphadenopathy and hepatomegaly. ¹

The first-line treatment for CML is ITK (except in the case of pregnancy). A short course of hydroxyurea may be given to symptomatic patients, and supportive care is necessary to manage complications related to ITK treatment. ⁷
Allogeneic stem cell transplantation may be considered for patients with chronic CML who do not respond to treatment with at least two different TKIs. ⁶

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BCR-ABL1: breakpoint cluster region/Abelson; TKI: tyrosine kinase inhibitor; ALL: acute lymphoblastic leukemia; AML: acute myeloid leukemia; CML: chronic myeloid leukemia; LSC: leukemia stem cells

References:

Argumentaire HAS. Évaluation de l’acte de recherche ou de quantification du gène de fusion BCR-ABL par RT-PCR dans le diagnostic et le suivi thérapeutique des leucémies myéloïdes chroniques et des leucémies lymphoblastiques aiguës. Novembre 2017.
Qu’est-ce que la leucémie myéloïde chronique ? Disponible sur lien : https://www.lmc-france.fr/la-lmc/qu-est-ce-que-la-lmc/ (consulté le 12/07/2023)
Orphanet. Leucémie myéloïde chronique. Disponible sur lien : https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=521 (consulté le 30/06/2023)
Monnereau A, Cornet E, Maynadié E, et al. Survie des personnes atteintes de cancer en France métropolitaine 1989-2018. Leucémie Myéloïde Chronique. Mars 2021.
Chomel JC, et al. Leucémie myéloïde chronique : Un modèle de dialogue entre la cellule souche leucémique et la niche hématopoïétique. Médecine/sciences. 2014; 30:452-61. DOI: 10.1051/medsci/20143004022
Ikhwan Rinaldi & Kevin Winston. Chronic Myeloid Leukemia, from Pathophysiology to Treatment-Free Remission: A Narrative Literature Review. Journal of Blood Medicine. 2023;261-277.
Hochhaus A, et al. European LeukemiaNet 2020 recommendations for treating chronic myeloid leukemia. Leukemia. 2020;34:966-984.