La leucémie aigue érythroblastique (LAM-M6) est une entité rare, représente 3 à 4% de l'ensemble des leucémies aigues. Il en existe deux types: l’érythroleucémie et la leucémie érythroïde pure. Elle se manifeste le plus souvent par des signes de cytopénie et d'infiltration des tissus extra-hématopoïétiques, elle est plus fréquente chez les adultes que chez les enfants et est de mauvais pronostic. Le but de notre travail est de mettre en évidence les particularités épidémiologiques, diagnostiques et évolutives de cette pathologie rare au sein du CHU HASSAN II de Fès. Nous rapportons le cas de sept patients diagnostiqués leucémie aigue érythroblastique LAM-M6 au laboratoire d'hématologie du CHU Hassan II de Fès entre Janvier 2009 et Aout 2013. Le diagnostic de leucémie aigue érythroblastique a été retenu sur un examen cytologique du frottis sanguin et du médullogramme ainsi que l'examen immunophénotypique. Il s'agit de deux adultes et cinq enfants, la plupart ont présenté une altération de l’état général, des signes de cytopénie et un syndrome tumoral. L’étude cytologique du frottis sanguin et du médullogramme ainsi que les résultats de l'immunophénotypage ont conduit au diagnostic de l’érythroleucémie chez six de nos patients et de leucémie érythroïde pure chez un seul patient. L’évolution a été différente pour ces patients. Le pronostic est grave d'où l'intérêt d'un diagnostic rapide et d'une prise en charge adéquate.
Macrophage activation syndrome (MAS) is a rare but potentially fatal disease. It is characterized by hyperinflammation, including the proliferation and activation of immune cells (CD8 T cells and NK cells) associated with hypercytokinemia. Patients present with fever, splenomegaly, and cytopenia, associated with a hemophagocytosis picture in the bone marrow. It can progress to a multiorgan failure syndrome (MODS), mimicking sepsis or a systemic inflammatory response syndrome (SIRS).We report the case of an 8-year-old girl admitted to the pediatric intensive care unit for the management of major trauma due to a domestic accident. She presented with a protracted fever in the context of a septic shock, despite appropriate treatment. The association with bicytopenia, hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia was suggestive of MAS which was confirmed by a bone marrow puncture showing hemophagocytosis. A Bolus of corticotherapy was then added to the supportive treatment and broad-spectrum antibiotherapy, with a good outcome.
Introduction: The discovery of hemolytic anemia must lead to a precise etiological assessment guided by clinical and biological data. The aim of our study is to describe the etiological profile of hemolytic anemia cases diagnosed in the hematology laboratory of the Hassan II University Hospital in FEZ. Material and Methods: We conducted a retrospective and descriptive study of hemolytic anemia cases diagnosed between January 2017 and July 2019 and based on epidemiological and clinical data collected from computerized reports and laboratory investigations. Results: The analysis of clinicobiological records identified 100 cases of hemolytic anemias. The mean age of our patients was 36 years , with a sex ratio (F /H) of 1.5. Anemia was symptomatic in the majority of patients. The etiologies found were : neoplasia in 32 patients, systemic lupus erythematosus in 10 patients , sickle cell disease in 10 patients, immunological thrombocytopenic purpura in 7 patients, glucose-6-phosphate dehydrogenase deficiency in 7 patients, alloimmunization in 5 patients, hemolytic uremic syndrome in 5 patients, hyperthyroidism in 5 patients, thalassemia in 4 patients, microspherocytosis in 4 patients, hypersplenism in 4 patients, paroxysmal nocturnal hemoglobinuria in 1 patient , pyruvate kinase deficiency in 1 patient, Gaucher disease in 1 patient, and the use of alpha-methyl Dopa in only one patient. Conclusion: Hemolytic anemia constitute a real diagnostic challenge. Neoplasia predominate in elderly subjects while autoimmune pathologies are more frequent in young subjects.
The plasmacytoid dendritic cells leukemia is a rare hematolymphoid neoplasm with a very poor prognosis, where skin involvement is common. It is a malignant proliferation developed at the expense of hematopoietic cells of the white blood cells, proximate to normal plasmacytoid dendritic cells. The phenotype of tumor cells initially described was CD4 + CD56 +, without T marker expression. CD56-forms have also been reported, even fewer without skin involvement. More specific markers are expressed by tumor cells: CD123, BDCA2, BDCA4. We report an unusual presentation of this entity, but without skin lesions. The diagnosis of plasmacytoid dendritic cells leukemia was made on the cytological and immunophenotypic data given. The patient refused treatment and left hospital against medical advice. He returned four weeks later to emergency in an array of septic shock, he died a few hours after admission. Discussion The nomenclature used to describe this entity (Blastic Dendritic Plasmacytoïd Cell Neoplasm (BPDCN) or derived leukemia of
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