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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. She received antibiotic therapy with Metronidazole. Five days later, she developed flaccid quadriparesis with mutism. Computerized tomography (CT) scan was normal, cerebrospinal fluid (CSF) examination was normal (there was no evidence of leukemic cells) and ophthalmological examination did not show any abnormalities. Brain MRI found hyperintense signal in the pons. The child improved without specific treatment, and clinical and complete neurological recovery was noted. This case highlights that myelinolysis can occur under some circumstances not related with hyponatremia such as malignancy, chemotherapy. [ABSTRACT FROM AUTHOR]

Still's disease, also known as systemic juvenile idiopathic arthritis (SJIA), and acute lymphoblastic leukemia have similar clinical and biological features posing diagnostic and treatment challenges. Indeed, while Still's disease is a diagnosis of exclusion in rheumatology, polyarthritis associated with hyperleukocytosis and fever, which is characteristic of this disease, are often detected in early stages of acute lymphocytic leukaemias. We here report the case of a 4-year-old girl, treated for Still's disease, in whom the diagnosis of acute lymphoblastic leukemia was made after 2 months, based on bone marrow biopsy. [ABSTRACT FROM AUTHOR]

Summary: T-cell acute lymphoblastic leukaemia is a highly heterogeneous and aggressive entity within haematological malignancies, marked by a poor prognosis, especially for refractory or relapsed cases. Their multiple and complex oncogenic processes make it difficult to identify effective targeted therapies for all patients. The most immature forms of the disease frequently contain alterations in epigenetic regulators that allow the identification of patients with a poor prognosis and eligible for treatment with hypomethylating agents. Furthermore, our recent work reveals that loss of function of the PRC2 polycomb complex is accompanied by a profound remodelling of the epigenetic landscape of blasts and confers synthetic lethality to bromodomain protein targeting. In the era of personalised medicine, understanding epigenetic deregulations in leukaemias allows the development of new effective targeted therapies for patients. [ABSTRACT FROM AUTHOR]

Résumé: Le gène RUNX1 code la sous-unité α du core binding factor , complexe transcriptionnel hétérodimérique impliqué dans la différenciation hématopoïétique. Les mutations et délétions germinales de RUNX1 sont responsables des thrombopénies familiales avec prédisposition aux leucémies aiguës myéloïdes (FPD/AML). Elles ont été décrites dans plus de 200 familles et l'on estime que plus de 6 000 seraient concernées par cette prédisposition génétique dans le monde. Le phénotype classique des individus porteurs de cette anomalie constitutionnelle inclut une thrombopénie d'intensité légère à modérée à plaquettes de taille normale, des anomalies des fonctions plaquettaires (associées ou non à une tendance hémorragique), une dysmégacaryopoïèse et une prédisposition au développement d'hémopathies malignes de phénotypes divers (leucémie aiguë myéloïde, syndromes myélodysplasiques, leucémie aiguë lymphoblastique T) pouvant survenir à tout âge. En cas de phénotype évocateur et de séquençage négatif, l'algorithme diagnostique devra inclure une recherche d'anomalie du nombre de copies du gène, afin de mettre en évidence une éventuelle délétion. La greffe de cellules souches hématopoïétiques est la seule thérapie curative mais implique des précautions pour la sélection des donneurs intrafamiliaux. Si la surveillance des individus indemnes d'hémopathie n'est pas encore complètement codifiée, il est conseillé de proposer un suivi hématologique régulier (hémogramme) complété d'une recherche d'anomalies moléculaires additionnelles annonciatrices d'une transformation maligne. The RUNX1 gene encodes for the alpha subunit of the core binding factor, a heterodimeric transcription factor complex involved in hematopoietic differentiation. RUNX1 germline mutations and deletions are implicated in the FPD/AML (familial platelet disorder with predisposition to acute myeloid leukemia). They have been described in more than 200 families and it is estimated that more than 6,000 families worldwide are affected by this genetic predisposition. The classical phenotype of individuals carrying this constitutional abnormality includes mild to moderate thrombocytopenia with normal volume, abnormal platelet functions (associated with a bleeding tendency or not), dysmegakaryopoiesis and predisposition to the development of hematological malignancies of various phenotypes (acute myeloid leukemia, myelodysplastic syndromes, acute T-cell lymphoblastic leukemia) at any age. When the phenotype is suggestive but sequencing remains negative, the diagnostic algorithm should include a copy number analysis to look for large deletions. Hematopoietic stem cell transplantation is the only curative therapy but implies precautions for the selection of intrafamilial donors. Monitoring of individuals who have not yet developed a hematological malignancy is not yet codified but it is recommended to provide a regular follow-up with complete blood count, associated with the screening of additional molecular abnormalities predicting a malignant transformation. [ABSTRACT FROM AUTHOR]

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Metastatic skin tumors may occur before or at any time before the diagnosis of malignancy. Skin metastases are thought to be indicative of advanced stage malignancy or non-treatment response. Cutaneous metastases are a worse prognostic mark, especially in patients with cancer of the lung, ovary, upper respiratory tract, or upper digestive tract. Acute myelomonocytic leukemia is a malignant-hematopoietic clonal sickness of bone marrow and disrupts production of normal blood cells. Although there are many and various dermatological findings in acute leukemia, they are mostly due to cytopenia and haemostasis disorders and skin metastasis is not frequent. Here, we present a case of skin involvement with a diagnosis of acute myelomonocytic leukemia, which is uncommon in the literature and nodules on the whole body.

Children and adolescents with acute leukemia have certainly a high chance of recovery, and much higher than adults, from the first line of treatment with conventional chemotherapy, but if a relapse occurs, subsequent survival remains poor, whether in lymphoblastic or myeloblastic subtypes. The accumulation of treatments is responsible for severe acute toxicities, long-term sequelae and a significant risk of secondary cancer. The exponential growth of new targeted therapies with small molecules, monoclonal antibodies and adoptive immunotherapy gives great hope for the most serious diseases. The large number of these new drugs raises the question of their respective place in new therapeutic strategies in the face of conventional chemotherapy and hematopoietic stem cell transplantation procedures, including in economic terms. [ABSTRACT FROM AUTHOR]

The number of childhood leukemia survivors increases. Many of them do not have any late effects and enjoy normal life. Others suffer from physical late complications or psychological, social and professional difficulties. The most common therapeutic risk factors for physical late effects are anthracycline, high-dose alkylating agents, brain irradiation and hematopoietic stem cell transplantation, notably after a total body irradiation-containing conditioning regimen. Inter-individual variability of late effects occurrence suggests that genetic susceptibility may play a role. The main late effects after leukemia treatment are endocrine (growth, gonadal function, fertility, and thyroid), nutritional (metabolic syndrome), cardiac, bone disorders and second malignancies. Interaction between physical late effects and quality of life are complex. [ABSTRACT FROM AUTHOR]

Management of childhood acute lymphoblastic leukemia, the most common cancer in childhood, is based on specialized oncology treatment teams, included in well-structured regional network. As soon as the diagnosis is established, the patient is hospitalized in a referral centre for childhood cancer, which works in close collaboration with community hospitals and has partnerships with other health care facilities to enable continuous and integrated care for patients and their families. The quality of patients cares rely heavily on the coordinated implementation of health professionals: medical doctors, dieticians, physiotherapists, psychologists, oncology nurse coordinators, and also social workers. [ABSTRACT FROM AUTHOR]

Allogeneic hematopoietic stem cell transplantation (HSCT) is a combination of replacement medicine (rather than surgery) and immunotherapy. Whatever the transplanted stem cell source (i.e. bone marrow, peripheral blood stem cells or cord blood stem cells), the graft provide both new hematopoiesis (erythrocyte, white blood cell and platelet-production) and new "immunopoiesis". This new infused immunologic system is able to participate in the underlying malignant disease control in association with previous chemotherapy and/or radiotherapy by providing graft-versus-leukelia effect. The main steps of HSCT are 1. Administration of a conditioning regimen, mainly myelo-ablative and then toxic in the frame of acute leukemia in children and adolescents; 2. Management of prolonged and severe neutropenia and of chemotherapy-related toxicities; 3. Management of possible immunological conflict between competent T-cells from the graft and recipient tissues (namely graft versus host disease); 4. Relapse occurrence monitoring and management; 5. Management of HSCT related late-effects and sequelae. [ABSTRACT FROM AUTHOR]

Weber christian's disease or idiopathic panniculitis is a rare condition characterized by an inflammation of the subcutaneous adipose tissue; it is a non-specific pathological condition that remains a central issue of debate and whose outcome is unpredictable. We here report the case of a 9- month-old female infant admitted in the hospital with sepsis preceded by respiratory symptoms and followed by the occurrence of small, hard, susceptible, asymmetrical erythematous subcutaneous nodes located in the arms and lower limbs. Cutaneous biopsy showed lobular hypodermitis with polymorphonuclear neutrophil infiltrations. The diagnosis of Weber Christian's disease was retained after eliminating other differential diagnoses. The patient was treated with prednisone with good outcome; during the follow-up period and during disease regression the diagnosis of B-cell acute lymphoblastic leukemia was made; this uncommon outcome has never been described in the literature. The aim of this study is to discuss any similar situation where another diagnosis was made in patients initially treated for idiopathic lobular panniculitis. This highlights the need for exhaustive etiological investigations and prolonged monitoring in order to search for a possible associated disorder. [ABSTRACT FROM AUTHOR]

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., Results: A total of 42 cases were collected, of which 45% suffered from acute lymphoblastic leukemia and 43% from acute myeloid leukemia. In 12% of cases, acute leukemia was not classified. The average age was 35 ± 15 years, with extremes of 19 and 72 years. 12% of the patients presented an age of poor prognosis. Comorbidities were present in 14% of patients. The deterioration in general condition was fairly constant with 95% of patients at WHO stages 3 and 4. All patients presented with bone marrow failure syndrome and tumor syndrome was found in 45%. Anemia and thrombocytopenia were present in almost all cases. Hyperleukocytosis at diagnosis was present in 28 patients (67%); among them 18 patients (64%) had leukocytes greater than 50 G/L. Death in hospital was found in 38% of patients and loss of sight in 31%. The median survival was 3 months. Survival was 30% at 6 months and 0% at 12 months., Conclusion: Acute leukemias are in our practice conditions of poor prognosis with a fairly short survival., (Copyright © 2023 SFMTSI.)

Introduction: Acute lymphoblastic leukemia (ALL) is the most common cancer in pediatrics. Immunophenotyping is an essential step for diagnosis and prognosis of acute lymphoblas- tic leukemias. Our objective is to determine the immunophenotypic profile of these acute leukemias in children followed in our university hospital and to look for possible aberrant phenotypes. Material and methods: This is a cross-sectional study of ALL cases that met the inclusion criteria, followed in the Pediatric Hematology-Oncology Unit of Hassan II University Hospital in Fez over a period of 5 years and 8 months and whose immunogenotyping was performed at the flow cytometry unit in the hematology laboratory of the same hospital. Results: Among 143 ALL cases collected,we selected 83 files. The immunophenotypic analysis allowed their classification in 2 categories: ALL B (84.3%) and ALL T (15.7%), the sex ratio H/F is 1.24 with male predominance in ALL T. The average age of patients is 6.3 ± 4.2 years, children over the age of 10 years are predominantly affected by ALL T. All patients had at least one abnormality of the blood count. It is anemia in 100% of cases, thrombocytopenia in 85.5% of cases and leukocytosis in 60% of cases. Pancytopenia was noted in 15.7 % of all ALL B patients. Circulating blasts were present in 86.6 % of cases. The study of the myelogram objectified a hypercellular marrow in 77,1% of the cases. All B cells expressed CD19, CD79a, CD22, IgM and CD20 markers. Expression of the CD10 immaturity marker was noted in 58 cases of ALL B (82.8%). All T-type blast cells expressed surface CD7 antigens and CD3 antigens. CD10 was found in 2 cases (15.4%) and absent in 11 cases (84.6%). Expression of myeloid antigen CD13 and/or CD33 was present in 49.4% of cases. Complete remission was obtained in 82.8% and 76.9% of ALL B and T respectively and the relapse was noted in 20.7% and 40% of cases successively. The death rate for all ALL in this series is 28.9%, (24.3% for ALL B and 53.8% for ALL T). Conclusion: The distribution of pediatric ALL in our study is similar to that of occidental series. The analysis of the clinical, biological and evolutionary characteristics of the different pheno- types of the patients is correlated with that of the literature. Comparing our data with those of other authors should help to better identify prognostic factors and improve survival. [ABSTRACT FROM AUTHOR]

Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography. The patient was treated with ciclosporin with rapid progression to acute myeloblastic leukemia. The progression of acquired amegakaryocytosis to acute leukemia is reported but it is generally not so rapid and above all it is preceded by myelodysplastic syndrome or medullary aplasia. This study highlights the importance of a close follow-up of these pathologies with a benign-like appearance. [ABSTRACT FROM AUTHOR]

Acute leukemias are rare disease in the neonatal period. They occur preferentially in newborns with trisomy 21. They often manifest as hepatosplenomegaly, extra-hematopoietic involvement and hyperleukocytosis. Blueberry Muffin Baby syndrome is observed in the neonatal period. Neonatal acute myeloïd leukemia (AML) is more common than acute lymphoid leukemia (ALL). Despite treatment, neonatal acute leukemias have a poor prognosis with a low percentage of overall survival. We report a case of neonatal AML on a ground of trisomy 21 revealed by a Blueberry Muffin Baby syndrome.

The aim of this study was to describe epidemiological, cytologic and immunophenotypic aspects of acute leukemias (AL) in children diagnosed at IBN SINA University Hospital Center and to determine the concordance between cytology and immunophenotyping results. This is a cross-sectional study conducted in the hematology laboratory of IBN SINA University Hospital Center between June 2012 and May 2014. Among the 104 cases with diagnosed AL, 52% were boys with a sex-ratio H/F= 1.32, the average age was 5.7 years. The distribution of different types of AL was: lymphoid AL (LAL) (74%), myeloid (AML) (20.2%), biphenotypic AL (BAL) (65.8%). Among the LALs, 78% were classified as B LAL and 22% as T LAL. Clinical signs were mainly presented with tumor syndrome (73.1%), fever (61%) and hemorrhagic syndrome (50%). The most common blood count abnormalities were: thrombopenia (89.4%), anemia (86.5%), hyperleukocytosis (79.8%). The rate of peripheral and bone marrow blasts was statistically higher for LAL than for AML and BAL (p <0.001). The rate of relapse and mortality was 21.2% and 16. 3% respectively. Concordance rate between the results of cytology and of immunophenotyping was 92.7% for LAL and 82.6% for AML. Diagnosis of AL is always based primarily on cytology. Immunophenotyping allowed us to make a better distinction between acute leukemias. The management of paediatric AL is a major health problem which requires specialized care centers. [ABSTRACT FROM AUTHOR]

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Résumé Les techniques de biologie moléculaire sont aujourd’hui devenues incontournables dans la prise en charge des leucémies aiguës (LA). Leur sensibilité élevée et les possibilités de standardisation en ont fait des outils de choix dans l’évaluation diagnostique et pronostique des malades, si bien qu’elles ont aujourd’hui leur place dans la plupart des protocoles en vigueur. 30 à 40% des leucémies aiguës présentent une translocation équilibrée induisant classiquement la fusion de deux gènes avec production d’un ARN chimérique, dont la mise en évidence constitue un argument diagnostique objectif et permet de définir certaines entités de LA au pronostic bien établi avec une prise en charge thérapeutique la plus adaptée possible. Au cours de ces dernières années, de nombreuses autres altérations ont pu être identifiées et trouvent particulièrement leur place en absence d’anomalie cytogénétique récurrente. Certaines sont d’ores et déjà étudiées de manière systématique. Enfin, la grande sensibilité de ces techniques leur a permis de s’imposer comme le gold standard pour l’évaluation de la réponse au traitement et/ou la détection des rechutes infracliniques, permettant une modification voire une intensification des traitements le plus précocement possible. Summary Molecular biology methods have become essential in the management of acute leukemia (AL). Their high sensitivity and opportunities for standardization made them tools of choice in diagnostic and prognostic evaluation and they currently have their place in most protocols. 30-40% of acute leukemia have a balanced translocation typically inducing fusion of two genes with production of a chimeric RNA, which constitute an objective diagnostic argument and define distinct AL entities. In recent years, many other alterations have been identified and they have a special place in absence of recurrent cytogenetic abnormality. Finally, the high sensitivity of these methods has allowed them to become a gold standard to assess response to treatment and/or to detect subclinical relapses, allowing treatment modification or intensification as early as possible. [ABSTRACT FROM AUTHOR]

Résumé Les leucémies aiguës, lymphoïdes et myéloïdes représentent 10 à 15% des hémopathies malignes et sont des affections rares qui en dehors de quelques formes sont souvent de pronostic péjoratif. Les leucémies aiguës myéloïdes (LAM) ont une incidence stable au cours du temps qui varie de 2,5 à 3,5/100 000 habitants/an dans les pays occidentaux. L’âge médian de survenue est de 63 ans. En dehors des formes avec anomalies cytogénétiques récurrentes, en particulier les LA promyélocytaires qui ont une survie à 5 ans voisine de 60%, les LAM ont une survie à 5 ans inférieure à 20% et qui ne s’améliore pas au fil du temps. Les leucémies aiguës lymphoblastiques (LAL) ont une incidence proche de 1,5/100 000 habitants/an. Elles sont surtout fréquentes chez l’enfant où l’incidence est supérieure à 6 avant 4 ans. Leur pronostic est meilleur chez l’enfant avec une survie à 5 ans de 90% chez l’enfant alors qu’elle n’est que de 35 à 40% chez l’adulte. On note une amélioration régulière de cette survie partout dans les pays occidentaux où les LAL B sont plus fréquentes et de meilleur pronostic que les LAL T. Summary Acute Leukemia are rare diseases that represent 10 to 15% of hematological malignancies and are often of bad prognosis. Incidence rate of Acute Myeloid Leukemia (AML) is stable along the time, varying between 2.5 to 3.5/100,000 inh/year in occidental countries. Median age was 63 years-old. AML with cytogenetic abnormalities, particularly promyelocytic forms have a 5-year survival rate close to 60% instead of what others that are more frequent, remained with a survival rate lower than 20%. Acute Lymphoblastic Leukemia (ALL) have an incidence rate of 1.5/100,000 inh/year. They are more frequent in children with an incidence rate up to 6 before 4-y-o. 5-year relative survival of ALL in children was close to 90% but was between 35 and 40% in adults. Survival increase slowly but regularly in all occidental countries. B-ALL are more frequent than T-ALL but are of better prognosis. [ABSTRACT FROM AUTHOR]

Pegaspargase (Oncaspar®), a pegylated form of native Escherichia Coli-derived L-asparaginase is an essential component chemotherapy used in the treatment of acute lymphoblastic leukemia (ALL) in pediatric and adult patients. Its particular toxicity profile requires a specific management to improve safety and tolerability and optimize treatment outcome and therefore survival. Within the framework of workshops of practice harmonization of the French Society of Children and Adolescent Cancers, diagnostic and management of the most commonly occuring toxicities (excluding coagulation abnormalities) during Pegaspargase treatment were reviewed according to the analysis of published studies., (Copyright © 2022 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Summary: Acute megakaryoblastic leukemia accounts for approximately 3–10% of acute myeloid leukemia in children. Its diagnosis may be difficult because of associated myelofibrosis. We report the case of a 7-month-old child who presented hepatomegaly with bicytopenia. She also developed bone and joint pain with recurrent aseptic arthritis. We suggested the diagnosis of megakaryoblastic leukemia early but multiple bone marrow investigations had been processed without positive results because of sampling problems and lack of abnormal cells in the morphological, phenotypic, and cytogenetic examinations. We had a variety of indirect evidence for our assumption: the x-ray showing periosteal new bone, lytic lesions and metaphyseal bands, bone marrow aspirate smears with micromegakaryocytes, and bone marrow biopsy suggesting myelofibrosis. This was very suggestive of leukemia but we could not prove it and we finally found megakaryoblasts on bone marrow aspirate smears after more than 2 months of investigation and initiated a course of corticosteroids. [Copyright &y& Elsevier]

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Summary: Sweet syndrome is a neutrophilic dermatosis that can lead to various inflammatory and neoplastic pathologies. We report a case of Sweet syndrome revealing acute leukemia at a 13-year-old girl, who had no history of illness. The diagnosis was made in spite of atypical skin lesions and was confirmed by the skin biopsy and the bone marrow examination. In spite of corticosteroid therapy and chemotherapy, the patient died. Sweet syndrome''s diagnosis requires an exhaustive etiologic survey. If there is no evidence of underlying disease, patients must be regularly monitored. [Copyright &y& Elsevier]

Summary: Predicting transfusion requirements relies both on epidemiology and therapeutic changes in hematology. The incidence rate of B-cell neoplasias especially non-Hodgkin lymphoma and myelodysplasia is increasing. Chemotherapy related myelodysplasia will reflect the improvement of solid tumor prognostic in the future. For myelodysplasias, therapeutic changes including oral iron chelators and more intensive transfusion policies will likely result in an increase of PRC requirements, a situation shared by sickle-cell disease. [Copyright &y& Elsevier]

The leukaemia with Denditric cells is a new entity of acute leukaemia co-expressing marker pens CD4(+) CD56(+), derived from the denditric plasmocytoïdes cells. It is about an extremely rare entity the first cases of which were described between 1994 and 1999 at patients presenting nodules and cutaneous tumors of red color made purple mostly spread. These first cases were initially reported under the term of lymphoma NK or of lymphoma blastic NK. The cutaneous infringement can be isolated at the beginning but an extra infringement cutaneous, mostly medullary, blood and ganglionic appears in every case after some months. The French Group of Study of Lymphomes Cutanés (GFELC) put the bases of this pathology in 1999 and gave him the temporary name of Hématodermie CD4(+) CD56(+). We consider now that this hématodermie derives from precursors of the denditric plasmocytoïdes cells. It was recently characterized by the Group of Immunological Study of Leukaemia (GEIL). It is at present mentioned leukaemia CD4(+) CD56(+) or leukaemia derived of denditric plasmocytoïdes cells (LpDC). If the circumstances of discovery and the clinical aspects of this entity were reported, there are still no criteria specific diagnoses of this leukaemia to iological plan (shot). We know however that the definition of this entity included the absence of expression of strong marker pens of the lineages myéloïdes, lymphoïdes B and T. We report here a case of LpDC discovered in the service of clinical haematology of CHU of Nice which was informed well as well on the clinical, biological and anatomopathological plan. [ABSTRACT FROM AUTHOR]

Acute myelogeneous leukemia is a rare disease (15-20% of acute leukemias in children). The treatment should be determined according to the risk factors. It is based on a very intensive chemotherapeutic regimen. Overall pronostic is currently less favorable than in ALL. However, 50-60% of children are cured with an adequate therapy. Pediatric AML is a complex and life-threatening disease, and optimal facilities are necessary for its diagnosis, treatment and health care. [ABSTRACT FROM AUTHOR]

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During the past few years, cytogenetic analysis of malignant proliferations contributed to the resolution of complex heterogeneous pathologic groups in smaller well defined entities. This is particularly true in the field of hematologic malignancies, for acute leukemia as well as for Lymphoma, Hodgkin’s disease or multiple myeloma. The improvement of knowledge regarding chromosomal or genetic rearrangements involved in the development of these tumors nowadays allows a better understanding of their pathological mechanisms and a better management of the patients. [Copyright &y& Elsevier]

Secondary leukemias group essentially together myelodysplastic syndromes and acute leukemias, therapy-related (chemo- or radio-), or consecutive to environmental factors. It’s now proven that some recurrent abnormalities are associated with effects of therapeutic agents, as –5/del(5q), –7/del(7q) linked to alkylating agents, or 11q23 and 21q22 abnormalities linked to inhibitors of Topoisomerase II. Even if important differences between secondary and “de novo” forms exist, the discrimination between these 2 categories is not always obvious: many common chromosomal abnormalities, “de novo” leukemias in older patients having characteristics close to those of postalkylating leukemias, neonatal forms possibly secondary to maternal affect. Recent studies identified some others chromosomal abnormalities in the secondary leukemias and confirmed the poor prognosis of these hemopathies. This review sums up criterions, circumstances and cytogenetic abnormalities. [Copyright &y& Elsevier]

The standard and molecular cytogenetic techniques now belong to the panel of mandatory analyses performed at diagnosis of acute leukemia. Chromosomal abnormalities contribute to define different types of leukemias and present the major advantage to be effective and independent prognostic factors, essential for therapeutic choices. Cytogenetic techniques allowing to identify hyperdiploi¨dy >50 chromosomes, t(12;21)(p13;q22)/TEL-AML1(ETV6-CBFA2), t(9;22)(q34;q11)/BCR-ABL, 11q23/MLL, t(15;17)(q22;q12–21)/PML-RARα, t(8;21)(q22;q22)/AML1-ETO and inv(16)(p13q22)/ CBFβ/MYH11 are developed. Among the techniques devoted to study genome, cytogenetics is a basic, simple and effective tool for giving a total picture of the genome through karyotype. Maintaining a systematic cytogenetic analysis is essential, not only because cytogenetics now belongs to routine practice but also because it still contributes to better defining morpho-immunologic sub-types of leukemia, to identify new cytogenetic entities and to understand hematopoiesis and leukemogenesis. [Copyright &y& Elsevier]

Granulocytic sarcoma is a rare tumor composed of immature cells of the granulocytic series wich usually occurs as a secondary manifestation of acute leukaemia. We report the case of a 60 years old woman without particular previous pathologies who was hospitalised for chronic diarrhea developed in a context of healt impairment state. The blood cell count revealed severe leucopenia and thrombopenia; an emergency right colectomy was accomplished. The histologic examination showed granulocytic sarcoma of the ascending colon. The death occurred rapidely as a consequence of a toxic shock.This observation seems to be the sixth case report of the granulocytic large bowel sarcoma in the literature which likely complicated a pre-existant and unknown myeloide leukaemia. [Copyright &y& Elsevier]

Résumé Objectif La détermination avec précision de la lignée cellulaire au cours de la leucémie aiguë (LA) est une étape cruciale dans le diagnostic et la conduite thérapeutique ultérieure. En Tunisie, faute de cytométrie de flux (CMF), certaines LA continuent d’être traitées sur la base de la cytologie seule. Notre objectif est de confronter les résultats de la cytologie à ceux de l’immunophénotypage et d’analyser les degrés de discordance. Patients et méthodes L’étude concerne 100 cas de LA. Elle consiste à effectuer une deuxième lecture a posteriori en double insu de lames de moelle de LA déjà diagnostiquées par deux cytologistes différents et de confronter aux résultats de l’immunophénotypage. Résultats Deux cas classés cytologiquement en LAM ont été reclassés à l’immunophénotypage en LAL T et leucémie aiguë biphénotypique. Trois cas classés cytologiquement LAL ont été reclassés leucémies aiguës indifférenciées (2 cas) et leucémie aiguë biphénotypique (1 cas). Quatre cas non identifiés à la cytologie ont été classés LAL B (3 cas), leucémie aiguë biphénotypique (1 cas). Dans les deux cas de discordance entre les deux cytologistes, la cytométrie de flux a permis de retenir le diagnostic de leucémie aiguë biphénotypique dans un cas et de LAM dans l’autre. Conclusion L’étude cytologique demeure insuffisante dans le diagnostic de lignée même avec des cytologistes expérimentés. L’immunophénotypage est un examen incontournable pour confirmer ou redresser le diagnostic de LA. Objective The determination of the cellular lineage in acute leukemia is a crucial step in the diagnosis and the later therapeutic conduct. In Tunisia, emerging country, some cases of acute leukemias are still treated on the basis of an only cytologic study because of lack of cytometry. Our objective is to realize a confrontation between cytology and flow cytometry in the diagnosis of AL and to analyze discrepancies. Patients and methods The study concerns 100 cases of AL. A second double-blind examination of the bone marrow smears of acute leukemias is realized by two cytologists and confronted to immunophenotyping. Results In two cases of AML, flow cytometry reassigned lineage into T ALL and biphenotypic AL. In three cases of ALL the lineage was reassigned into undifferentiated acute leukemia (2 cases) and biphenotypic acute leukemia (1 case). Lineage was not established in four cases, immunophenotyping allowed the diagnosis of B ALL in 3 cases, and of biphenotypic acute leukemia in 1 case. In both cases of discrepant findings, flow cytometry allowed the diagnosis of biphenotypic acute leukemia in a case and of AML in the other one. Conclusion The cytological study remains insufficient in the diagnosis of lineage even with experimented cytologists. Immunophenotyping is essential in lineage assignment and reassignment. [ABSTRACT FROM AUTHOR]

The authors report the case of a patient with acute leukaemia (AL) CD4+/CD56+ considered as the rare case of AL. These cells CD4+/CD56+ result from a lymphoid transformation related to the dendritic cells plasmocytoïdes. The clinical picture is the infiltrated cutaneous lesions associated a swollen glands syndrome. The diagnosis is suspected by cytology analysis and confirmed by immunophenotype and cytogenetic which finds a massive infiltration medullary blastic which expresses CD4+ and CD56+, the negativity of the markers T, B and the myeloid markers and cytogenetic abnormalities 5, 9 and 15. Whatever type or regimen of chemotherapy and radiotherapy is tried, the outcome remains dismal. The relapses are frequent and are especially in skin, marrow and central nervous system. Themedian survival at 1 year was 52% for the patients treated by polychemotherapy and 60% after allogenic bone marrow transplantation. Presently, no therapies have demonstrated remissions for 5 years or more. [ABSTRACT FROM AUTHOR]

Abstract: Acute leukemia is uncommon in neonates and has a much poorer prognosis than in older children. We report on a case of acute lymphoblastic leukemia observed in a neonate who had bleeding and hepatosplenomegaly at birth, which justified intensive care during the first postnatal week. Despite early appropriate treatment, the patient died at 7 months of age. We present here physical and laboratory findings, which indicate a grim prognosis. These criteria should be considered carefully in order to ensure a realistic information for the parents and appropriate decisions. [Copyright &y& Elsevier]

Abstract: Introduction: Ostéoarticular manifestation whose reveal leukaemia in 4% of the cases, regress completely with haematological remission. Exegesis: We report two observations of leukaemia revealed by polyarthritis. A 22-year-old woman has presented a polyarthritis 8 months before de diagnosis of acute leukaemia. A 34 years old men, has presented one month before admission an acute polyarthritis revealing chronic myeloid leukaemia. Conclusion: Polyarthritis may reveal an acute or chronic leukaemia. Systematic blood analysis can make a difference in diagnosis of recent polyarthritis. [Copyright &y& Elsevier]

For two decades, the prognostic of adult patients with ALL was improved based on pediatric-inspired protocols. These approaches based on less myelosuppressive drugs have led to improved response rates, decreased relapse rates, with a benefit in survival observed in patients aged up to 50-60-years-old. Therapeutic intensification came with a decrease in the use of allogeneic hematopoietic stem cell transplantation, with current indications mainly based on the level of measurable residual disease. Pediatric approaches are however limited in older patients or in patients with comorbidities, who are at greater risk to develop adverse effects especially to asparaginase. Future progresses will arise from personalized medicine including targeted therapy in some ALL oncogenic subgroups and immunotherapy. Monoclonal antibodies, bispecific antibodies, antibody drug conjugates and CAR-T cells have shown encouraging results in relapsed/refractory diseases. These strategies are now evaluated frontline in children and adults to further increase the quality of response, to limit the toxicity of treatments including allogeneic transplant. The objective of this review is to discuss the benefit and the limits of pediatric therapeutic strategies in adults and the perspectives offered by new approaches including immunotherapies., (Copyright © 2020 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Microtransplantation (MT) is based on injection of HLA-mismatched G-CSF mobilized hematopoietic stem cells, in combination with chemotherapy but without use of conditioning regimen nor immunosuppressive drugs. As a result, a transient microchimerism is induced without engraftment. Its efficacy relies both on host immune system stimulation (recipient versus tumor) and on a graft versus tumor effect. Data are scarce and concern mostly Asian patients with acute myeloid leukemia (AML) and high risk myelodysplastic syndrome (HR-MDS). In comparison to conventional treatment without MT, higher complete remission rates and longer disease free survival and overall survival have been reported. Safety seems acceptable. The most frequent adverse event is non-severe cytokine release syndrome. Risk of GVHD remains very low. Here, we summarize the published data and detail the practical aspects of the procedure. Current data are not strong enough to provide recommendations on indications. Nevertheless, it seems reasonable to propose MT to patients with AML or HR-MDS, regardless of age, presenting an indication for allogeneic stem cell transplantation but ineligible for it. MT is still under investigation and rather be proposed within clinical trials., (Copyright © 2020 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Serous degeneration of bone marrow is a rare hematological complication, of multiple etiologies, which physiopathology is yet to be fully understood. It is described as a focal hypoplasia of the bone marrow and atrophy of its adipocytes coupled with an extracellular deposit of an eosinophilic substance that corresponds to "hyaluronic acid". The prognosis of these lesions depends on the etiological diagnosis., Observation: We report the case of a 19-year-old female patient without no notable pathological history, hospitalized in a regional hospital for mucocutaneous pallor with an impaired general condition. The biological assessment revealed a pancytopenia, and the presence of 60 % blast cells on the blood smear, which was in favor of acute leukemia. As part of the etiological assessment, a bone marrow biopsy was performed, revealing a typical aspect of serous degeneration. The bone marrow cellularity was reduced, made of rare hematopoietic cells, dissociated by pale extracellular deposits that were colored by PAS and alcian blue, with no individualized blasts., Discussion: Serous transformation of the hematopoietic marrow is a rare condition, described at the end of the 19th century. The most severe forms are observed in young patients with hypoplasia. It most often manifests as pancytopenia at the beginning, if not then by an isolated cytopenia, anemia or thrombocytopenia. It is interesting to underline the contribution of MRI in diagnosis. The foci of bone marrow serous degeneration are characterized in the long bones by a fluid signal with a T1 hypo-signal and a T2 hyper-signal with no enhancement after injection of gadolinium, unlike bone metastases. The histology of bone marrow biopsy finds focal marrow hypoplasia of the hematopoietic lines associated with a depletion of adipocytes and an eosinophilic gelatinous interstitial infiltration alcian blue and PAS positive. The histological differential diagnosis is amyloidosis due to red Congo positivity, a previous biopsy (the absence of adipose tissue or hematopoietic tissue with granulation tissue and bone formation) and interstitial edema. Bone marrow necrosis can also be observed. Serous degeneration of bone marrow can be a symptom of a chronic systemic illness. In most cases, it is found in cases of severe chronic malnutrition, such as anorexia nervosa, certain cancers (acute leukemia), systemic diseases (lupus) or severe infection. Though there is no specific treatment for this complication, the treatment of the cause most often allows for the complete regression of the peripheral and spinal anomalies., Conclusion: In rare cases, as is the case in our observation, the serous degeneration of bone marrow can reveal underlying solid tumors or hemopathies, knowledge of it can help guide the investigations towards these etiologies., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

A 27-year-old woman, presented to the Emergency Department with swelling of breasts, twelve days after childbirth. Physical examination found a huge tumefaction of breasts, without inflammatory sign or nipple discharge. The patient was put under anti edematous while waiting for the Breast ultrasound. After 35 days, she had a dyspnoea (stage IV) without chest pain. Then, physical examination finds swollen breasts, bilateral cervical lymphadenopathy, one axillary node, inguinal left lymphadenopathy, and gingival hypertrophy. Acute leukemia is diagnosed. Acute leukemia is a cancer of hematopoietic stem cells, characterized by clonal proliferation of hematopoietic precursor cells. The result is a hypertrophy of hematopoietic organs: lymphadenopathies, hepatomegaly, and splenomegaly are frequent, but no case of breast hypertrophy has been described. We report an historical case of acute leukemia revealed by bilateral breast tumefaction. [ABSTRACT FROM AUTHOR]

CAR-T cells are genetically modified human lymphocytes and gene therapy medicinal products. They are developed to treat cancers that express a membrane antigen targeted by the CAR. The FDA approved the two first-in-class medicinal products in 2017 and EMA in August 2018; both are autologous CAR-T cells targeting CD19 that is expressed at the surface of normal B-cells throughout their differentiation, and on B-cell lymphoid malignancies. Clinical efficacy was demonstrated for B-cell acute lymphoblastic leukemias, non-Hodgkin's lymphoma and chronic lymphocytic leukemia, although the marketing authorizations are less liberal in terms of indications. Manufacturing of these personalized treatments necessitates that a novel organization and supply chain be set in place, to ensure product preservation, patient safety and compliance with complex regulatory requirements. Side effects are commensurate with clinical efficacy and can be life-threatening: proper management imposes tight coordination between various specialists, particularly between hematologists and intensive care practitioners. High pricing for these treatments is part of a long-term trend for increasing costs of innovations in hematology and oncology; it questions the ability of healthcare systems to sustain their reimbursement., (Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)