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Research Article | DOI: https://doi.org/10.31579/2639-4162/247
1Laboratory Department of the National Hospital Center of Nouakchott.
2National Oncology Center.
3Faculty of Medicine, Pharmacie and Dendistry of Nouakchott, Mauritania.
*Corresponding Author: Mohamed Lemine Salem, Laboratory Department of the National Hospital Center and Faculty of Medicine, Pharmacie and Dendistry of Nouakchott, Mauritania.
Citation: Abbah M. Mbareck, Jiddou Abdou, Mohamed L. Salem, (2025), Chronic myeloid leukemia in Nouakchott: epidemiological, clinical, cytological and therapeutic aspects: about 50 cases, J. General Medicine and Clinical Practice, 8(3); DOI:10.31579/2639-4162/247
Copyright: © 2025, Mohamed Lemine Salem. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Received: 23 January 2025 | Accepted: 10 March 2025 | Published: 25 March 2025
Keywords: chronic myeloid leukemia; myeloproliferative neoplasms; Philadelphia chromosome
Introduction: Chronic myelogenous leukemia (CML), a rare myeloproliferative neoplasms (MPNs) representing 2 to 5% of childhood leukemias and 15% of adult leukemias, according to the 2008 WHO classification and its 2016 update, it constitutes one of the preferred study models of leukemogenesis because tumor cells are characterized by an exchange of chromosomal material: the t (9;22) translocation, which leads to the formation of an abnormal chromosome 22, called the Philadelphia chromosome (Ph). The translocation leads to the formation of the BCR-ABL fusion gene.
Material and method: This are a retrospective study with analytical purposes, spread over 5 years (2018-2022), on 50 cases of CML, diagnosed and followed in Nouakchott.
Results: a male predominance with a sex ratio of 1.08, the average age was 43 years with extremes between 18 years and 69 years, splenomegaly was present in 94%, anemia was present in 34% of patients. the average white blood cell count was 169,364/ mm3, all patients presented myelemia associated with PMNs in the blood smear, the search for the Philadelphia chromosome (BCR/ABL) was positive in all patients, the evolution was favorable in 64%, death occurred in 12%.
Conclusion: The diagnosis of CML, although it is always mentioned on the clinical arguments (age, splenomegaly) and haematometric (hyperleukocytosis and myelemia), currently requires to be confirmed, either by the search for the Ph chromosome in cytogenetics, which allows in addition to detecting additional chromosomal anomalies or by FISH method which also makes it possible to detect cases of masked Ph and deletions of ABL, or by molecular biology by qualitative RT-PCR method allowing to detect with certainty the transcript of BCR/ABL.
Chronic myelogenous leukemia (CML), a rare myeloproliferative neoplasm (MPNs) representing 2 to 5% of childhood leukemias and 15% of adult leukemias. according to the 2008 WHO classification and its 2016 update [1,2]
CML is one of the preferred models for studying leukemogenesis because the tumor cells are characterized by an exchange of chromosomal material: the t (9;22) translocation, which results in the formation of an abnormal chromosome 22, called the Philadelphia (Ph) chromosome. The translocation leads to the formation of the BCR-ABL fusion gene. In vitro and in vivo experiments have demonstrated that the Bcr-Abl protein, through its deregulated tyrosine kinase activity, is responsible for the disease.[3].
Indeed, the chimeric protein, encoded by the BCR-ABL fusion transcript resulting from this rearrangement, has a constitutively deregulated tyrosine kinase activity and is directly responsible for leukemic transformation.
In the absence of treatment, CML progresses within 3 to 5 years to a rapidly fatal acute leukemia. Treatments such as hydroxyurea or busulfan have very little effect on patient survival. Allogeneic bone marrow transplantation can cure patients but can only be offered to a limited number of patients. Interferon alpha (INF-α) has improved the survival of responding patients but these are few in number and the side effects have made its use limited. Today, imatinib mesylate, the first tyrosine kinase inhibitor specific to the Bcr-Abl protein, has become the first-line treatment for this blood disease, making CML an example of a blood disease requiring targeted therapy.
The long-term results of this new drug will determine the clinician's attitude towards allogeneic bone marrow transplantation, which is, until today, considered the only curative treatment.[4.5]
The diagnosis of CML, although it is always suggested on the basis of clinical (age, splenomegaly) and hematometry (hyperleukocytosis and myelemia) arguments, currently needs to be confirmed, either by the search for the Ph chromosome in cytogenetics, which also allows the detection of additional chromosomal abnormalities (ACA) or by FISH method which also allows the detection of cases of masked Ph and ABL deletions, or by molecular biology by qualitative RT-PCR, a method allowing the detection with certainty of the bcr/abl fusion transcript. Indeed, there are rare cases of so-called atypical CML not having the Ph. Moreover, it constitutes the biological data essential for monitoring the response to treatment with ITKs, which is based on the cytogenetic response which can be assessed using conventional karyotype or FISH and the molecular response requiring real-time RT-PCR, the most precise parameter for assessing residual disease.
The objective of our study is to highlight the epidemiological, clinical, hematological and cytogenetic characteristics of chronic myeloid leukemia in patients with CML followed in Nouakchott between 2018 and 2022.
Our samples were collected at the National Hospital Center (CHN) and the National Oncology Center (CNO). Patients come from all medical facilities in the city of Nouakchott: private facilities, outpatient clinics and hospitalization services of the various hospitals. Our study spanned a five-year period from 2018 to 2022.Our study was retrospective. Data were collected from patient records. The study population was all adult patients of both sexes. All patients with confirmed CML were included in our study.
Over a period of 5 years; from 2018 to December 2022, we collected 50 cases of chronic myeloid leukemia meeting the inclusion criteria of our study. The number of male patients was 26 or 52%, compared to 24 female patients or 48%. That is a sex ratio of 1.08. The average age was 43 years with extremes between 18 and 69 years.
Clinical examination
| Splenomegaly | Present | Absent |
| Number | 47 | 3 |
| Percentage | 94% | 6% |
Table I: Distribution of patients according to the presence of splenomegaly
Paraclinical examination
Blood count
Hemoglobin
| Hb (g/dl) | Anemia | Normal | Total |
| Numbers | 17 | 33 | 50 |
| Percentage (%) | 34% | 66% | 100% |
Table II: Distribution of patients according to the presence of anemia
| WBC (103/ul) | <50> | 50-150 | >150 |
| Numbers | 2 | 30 | 18 |
| Percentage (%) | 4% | 60% | 36% |
Table III: Distribution of patients according to white blood cell count
Blood smear
100% of patients had myelemia associated with mature neutrophils.
Philadelphia chromosome (BCR/ABL) search:
In our study, all of our patients benefited from BCR/ABL testing which came back positive, i.e., 100%.
Ultrasound:
19 of our patients, or 38%, had an abdominal ultrasound scan that revealed splenomegaly; the remaining 31 patients, or 62%, did not have an abdominal ultrasound scan.
Treatment:
Figure 1 : Distribution of patients according to treatment
Figure 2 : Distribution of patients according to evolution-64% of patients had a favorable evolution and 16% were lost to follow-up, 12% died and 8% were evacuated at their request, outside.
Epidemiological aspects
Sex
In our study we have a male predominance with a sex ratio M/F of 1.08, which agrees with the data in the literature [6] and a study carried out in Niger with a sex ratio equal to 1.6 [8]. whereas in a study carried out in Morocco the female predominance was more marked with a sex ratio M/F of 0.72 [7].
The Age
| Series | Country | Average Age (years) |
| Nacer Redhouane and al.[9] | Algeria | 43.5 |
| Elias Jabbour and al.[10] | United States | 54 |
| Segbena and al.[11] | Togo | 40.5 |
| Corm and al.[12] | France | 56 |
| SH Allah and al.[7] | Morocco | 46.52 |
| Our series | Mauritania | 43 |
Table IV: Comparison of mean age with different studies
The average age in our study was above that of the study in Togo and close to that of the Algerian study, lower than that reported in Moroccan, French and American studies.
Clinical aspect
Splenomegaly
It was found in 47 patients, or 94%, which is higher compared to studies carried out in Morocco (82.6%) and Algeria (90%).[7.13], while in other studies it was found at 100%[8,14,15]
Paraclinical aspects
Blood count
The blood count is the most important test because it alone allows the diagnosis to be made. Hyperleukocytosis in CML is pronounced, greater than 20,109 leukocytes/L, mainly composed of polymorphonuclear neutrophils, associated with basophilia and eosinophilia. Myelemia is constant and harmonious, without a hiatus in differentiation, and blastosis is low in the chronic phase (<5>
| Series | Average WBC rates (/mm3) |
| Segbena et al.[11] | 188710 |
| Mukiibi et al.[18] | 223700 |
| Kohobo[19] | 158000 |
| El Mouhidi[20] | 239000 |
| JamalEddine et al.[17] | 196900 |
| SH. Allah et al.[7] | 106111 |
| Our series | 169364 |
Table V: Comparison of mean WBC rate in our series and other studies
Blood smear
Myelemia was found at a rate of 100%. Myelemia (i.e. the passage into the blood of myeloid cells at all stages of differentiation) is constant, without a hiatus in differentiation, representing 10 and 50% of the elements[21], consisting of metamyelocytes, myelocytes and some promyelocytes and more rarely myeloblasts, this result is consistent with that of the different series to which our study was compared.
Philadelphia chromosome (bcr/abl)
In our series, 100% of patients in whom the cytogenetic study was carried out had a PHL chromosome, which is identical to the percentage found by Moroccan authors.[7].
| Series | Percentage of beneficiaries | BCR-ABL positive |
| Segbena et al. [11] | 100% | 100% |
| Kohobo [9] | 7% | 100% |
| El Mouhidi [20] | 3.7% | 100% |
| JamalEddine et al. [17] | 100% | 100% |
| SH. Allah et al.[7] | 52% | 100% |
| Our series | 100% | 100% |
Table VI: BCR/ABL positivity rates according to different studies
Therapeutic aspects
In our series 47 patients or 76% were on Imatinib 400mg and 3 patients or 14% on Hydrea 500mg.
Evolution
Six patients (12%) in our series died, all of these cases were due to accumu- lation or serious hematological and septic complications. This rate remains low compared to other series (Table VII).4 patients (8%) evacuated outside following their request.
Note that 8 (16%) patients were lost to follow-up, which suggests that the death rate may be higher.
| Series | Number of patients | Number of deaths | Death rate (%) |
| Ongoren et al.[23] | 21 | 4 | 19.04% |
| Quintas-Cardama et al.[24] | 23 | 7 | 30% |
| Garg et al.[25] | 34 | 4 | 8.33% |
| JamalEddine et al. [22] | 10 | 0 | 0% |
| SH. Allah et al.[7] | 114 | 6 | 5.25% |
| Our series | 50 | 6 | 12% |
Table VIII: Comparison of death rates between our series and other studies
Chronic myeloid leukemia is a malignant haemopathy belonging to the group of myeloproliferative neoplasms (MPS) according to the WHO 2008 classification and its 2016 update, representing 2 to 5% of childhood leukemias and 15% of adult leukemias.
The aim of this study was to analyze the epidemiological, clinical, cytological and therapeutic aspects of chronic myeloid leukemia monitored at the National Hospital Center (CHN) and the National Oncology Center (CNO). During this study a predominance of the male sex was noted, with a sex ratio of 1.08, the average age of our population was 43 years with extremes between 18 and 69 years. The presence of splenomegaly was clearly marked in 47 patients or 94%. The blood count revealed anemia in 17 patients, or 34%.
All patients in our series, i.e., 100%, had hyperleukocytosis with PMNs predominance on the blood count.
All patients had myelemia associated with mature PMNs on blood smear. Philadelphia chromosome testing was positive in all patients. The evolution was favorable in 64% of patients and the death rate was 12%.
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