Leucemie

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Neonatal acute myeloid leukemias (AML) occurred within the first 28 days of life and constitute only a small proportion of all AL. They are distinguished from leukemias of older children by their clinical presentation, which frequently includes cutaneous localizations ("blueberry muffin rash syndrome") and a leukocytosis above 50 ×109 /L. This proliferation may be transient, causing a transient leukemoid reaction in a background of constitutional trisomy 21 ("Transient Abnormal Myelopoieseis" or TAM) or Infantile Myeloproliferative Disease in the absence of constitutional trisomy 21 ("Infantile Myeloproliferative Disease" or IMD)...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

TYROSINE KINASE INHIBITORS FOR CHRONIC MYELOID LEUKEMIA. About 20 years ago, the discovery of imatinib, the first ATP-competitive inhibitor of BCR::ABL1 the driving oncoprotein of chronic myeloid leukemia (CML), revolutionized patients' outcome by transforming a fatal condition into a chronic one. Today, five more tyrosin kinase inhobitors (TKIs) have been approved, allowing to some extent individualization of drug therapy. The main therapeutic objective is to protect patients from progression towards a fatal blast crisis and to restore of a near-to-normal life expectancy on lifelong TKI treatment...

The spectrum of childhood leukemia predisposition syndromes has grown significantly over last decades. These predisposition syndromes mainly involve CEBPA, ETV6, GATA2, IKZF1, PAX5, RUNX1, SAMD9/SAMD9L, TP53, RAS-MAPK pathway, DNA mismatch repair system genes, genes associated with Fanconi anemia, and trisomy 21. The clinico-biological features leading to the suspicion of a leukemia predisposition are highly heterogeneous and require varied exploration strategies. The study of the initial characteristics of childhood leukemias includes high-throughput sequencing techniques, which have increased the frequency of situations where a leukemia predisposing syndrome is suspected...

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No abstract text is available yet for this article.

INTRODUCTION: Acute leukemia is both a diagnostic and therapeutic emergency. Our study aimed to describe the prognostic factors and survival of adults with acute leukemia in Burkina Faso. PATIENTS AND METHODS: Cross-sectional descriptive study with retrospective data collection covering a period of 4.5 years (2018-2022) in two university hospitals in Burkina Faso. Were included all patients over 18 years hospitalized for acute leukemia in these sites with a usable medical record...

The treatment of chronic myeloid leukemia relies on orally available tyrosine kinase inhibitors targeting the BCR::ABL1 oncoprotein. Bosutinib is a second generation adenosine triphosphate-competitive inhibitor approved for use in frontline adult chronic phase-chronic myeloid leukemia and all phases-chronic myeloid leukemia in the second line setting or beyond. Its efficacy was demonstrated in several pivotal clinical trials at 400mg once daily in the first line context and at 500mg once daily beyond first line...

Tyrosine kinase inhibitors (TKI) have emerged as a paradigm-shifting therapeutic approach for the treatment of chronic myeloid leukemia (CML) following their regulatory approval in 2001. These agents have revolutionized the management of CML by significantly improving patient outcomes and enabling them to achieve near-normal life expectancies. Consequently, the utilization of TKI has become increasingly prevalent, accompanied by the recognition and management of their associated adverse effects. Given the expanding patient population receiving TKI therapy, it is imperative that hematologists, as well as general practitioners, assume the responsibility of closely and meticulously monitoring patients' treatment progress while effectively addressing the occurrence of any untoward effects...

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No abstract text is available yet for this article.

CAR-T cell therapy for patients with hematological malignancies has been practiced at the Basse-Normandie Hematology Institute since November 2022. This treatment requires the care pathway to be coordinated by the nurse coordinator. Nurses play a key role in the early diagnosis of side effects induced by this drug. Interdisciplinary collaboration and the value of teamwork are also emphasized.

In this case report we present a feline large granular lymphocyte (LGL) lymphoma, a rare morphologically distinct subtype of lymphoma, in a twelve-year-old female spayed domestic short hair cat, with high suspicion of leptomeningeal lymphomatosis due to magnetic resonance imaging findings and results of cerebral spinal fluid analyses. Diagnosis of LGL lymphoma was confirmed by means of blood cytology and polymerase chain reaction for antigen receptor rearrangements.

Juvenile myelomonocytic leukemia (JMML) and myelodysplastic syndromes (MDS) of children are rare and aggressive diseases. They both have the particularity of being very frequently associated with an underlying predisposition syndrome, which must be systematically investigated by meticulous clinical exam completed by molecular analysis on fibroblasts, in order to guarantee the best therapeutic management. New generation sequencing techniques have made it possible to better define the landscape of constitutional predisposing pathologies, to understand the clonal evolution that leads to the development of hematological malignancies and to identify new prognostic markers...

Since 2020, a promising new treatment has been offered to elderly patients with acute myeloid leukemia. However, adverse events complicate their treatment, which is performed on an outpatient basis. The advanced practice nurse could provide assistance in the follow-up of these elderly and polypathological patients, who require regular clinical and biological monitoring, adaptation of their therapies and the establishment of city-hospital coordination likely to guarantee their maintenance at home.

Systemic inflammatory or autoimmune diseases (SIAD) are observed in up to a quarter of patients with myelodysplastic syndromes (MDS) or chronic myelomonocytic leukemia (CMML), with a broad clinical spectrum including asymptomatic biological abnormalities, isolated inflammatory clinical manifestations (recurrent fever, arthralgia, neutrophilic dermatoses…) or identified systemic diseases (giant cell arteritis, recurrent polychondritis…). Recent advances in molecular biology have shed new light on the pathophysiological mechanisms that link inflammatory manifestations and myeloid hemopathies, particularly in VEXAS syndrome following the identification of somatic mutations in the UBA1 gene, or in neutrophilic dermatoses with the concept of myelodysplasia cutis...

Central pontine myelinolysis is a demyelinating disorder mainly affecting the central pons. In some cases, it is associated with extrapontine myelinolysis. It is usually caused by rapid correction of hyponatremia and osmotic shock. We here report the case of a 3.5-year-old girl diagnosed with acute lymphoblastic leukemia admitted to our Oncology Unit with neutropenic fever and diarrhea. Laboratory tests showed mild neutropenia, normochromic normocytic anemia. Electrolyte tests were normal without hyponatremia...

INTRODUCTION: Complete remission (CR) in patients with acute myeloid leukemia (AML) is still morphologicaly defined, thus corresponding to a wide range of tumor burden. OBJECTIVES: we aimed to evaluate the residual disease (MRD) status in patients with AML, as well as perform a molecular analysis of the FLT3/ITD gene in patients with normal karyotype. MATERIAL AND METHODS: adult patients with AML, diagnosed according to the WHO 2016 criteria, were included...