Background/aim: Hemophagocytic lymphohistiocytosis (HLH) is a clinical, biological, and pathological entity that is rare but has certain morbidity that may be life-threatening. This work aims to establish a focus on the hemophagocytic lymphohistiocytosis and analyze different aspects of diagnosis while emphasizing the biological data. Materials and methods: We report the results of a retrospective study conducted in the hematology department of Avicenna Hospital in Marrakesh. Thirty-one patients with hemophagocytic lymphohistiocytosis were enrolled. Results: The clinical presentation was dominated by fever and deterioration of the general state for almost all our patients. Splenomegaly was objectified in 90% of the patients. Hepatomegaly, lymphadenopathy, and hemorrhagic manifestations were observed in almost 50% of the patients. Biological assessments revealed bi-or pancytopenia in 96% of the patients, and coagulation disorders in 51% of the patients. On the other hand, hyperferritinemia was found in 84% of the patients, and hepatic cytolysis and hypertriglyceridemia in half of the patients. Hemophagocytosis was observed in all bone marrow samples taken from our patients. Concerning the evolution of patients, in 38.5% of the patients, the evolution was favorable with regression of clinical and biological signs. Twenty six percent of the patients had died, mainly from multiple organ failure and disseminated intravascular coagulation. Conclusion: HLH is a diverse condition with many causes and is likely to be under-recognized, which contributes to its high morbidity and mortality. Clinicians need to be able to recognize the signs and symptoms commonly seen in HLH and actively pursue this diagnosis in the cases of undiagnosed febrile illness with multiorgan dysfunction. Early recognition is crucial for any reasonable attempt at curative therapy to be made.
Objectifs : Les thalassémies sont la conséquence d'un déséquilibre de synthèse entre les chaînes ␣ et  de l'hémoglobine. Ce sont des anémies hémolytiques héréditaires, qui présentent un problème de santé publique vue leur fréquence et les difficultés de leur prise en charge. L'objectif de ce travail est d'étudier le profil épidémiologique, clinico-biologique, thérapeutique et évolutif des patients thalassémiques au Maroc. Méthodes : Nous rapportons les résultats d'une étude rétrospective, multicentrique, portant sur tous les cas de thalassémies suivis au niveau des trois hôpitaux : hôpital militaire Avicenne de Marrakech, CHU Mohammed VI de Marrakech et le centre régional de transfusion sanguine d'Agadir. Résultats : Durant cette période nous avons colligé 81 cas de thalassémies. L'âge moyen était de 13 ans (1 mois et 60 ans) avec un sexe ratio (M/F) de 0,8. Le motif de consultation était dominé par le syndrome anémique dans 43 % des cas. L'électrophorèse de l'hémoglobine avait objectivé une prédominance de la forme -thalassémie mineure. Sur le plan évolutif, la surcharge en fer et les complications endocriniennes étaient les plus fréquentes. Conclusion : Le développement d'un programme de prévention reposant essentiellement sur l'éducation sanitaire, la diminution des mariages consanguins et le conseil génétique, sont probablement les voies principales de lutte contre l'apparition de nouveaux cas thalassémiques.
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