Neonatal leukemia and Blueberry Muffin Baby Syndrome: About a case of trisomy 21 / Leucémie néonatale et Blueberry Muffin Baby syndrome : à propos d'un cas de trisomie 21

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.

[Macrophagic activation syndrome revealing Hodgkin lymphoma: an individual case]. / Syndrome d'activation macrophagique révélant un lymphome hodgkinien : à propos d'un cas.

Macrophagic activation syndrome (SAM) or Hemophagocytic lymphohistiocytosis is a clinical-biological entity of non-specific proliferation and activation of macrophages of the reticulohistiocytic system, with phagocytosis of the figurative elements of blood. It is a rare pathology combining non-specific clinical and biological signs and images of hemophagocytosis. It can be primary or acquired secondary to an infection, an autoimmune or neoplastic disease. While T or NK lymphomas are the classic causes of reactive SAM and its association with Hodgkin lymphoma is exceptional. This is a diagnostic and therapeutic emergency given the risk of development towards a fatal multivisceral failure due to an absence of appropriate support. This is a report of a case of SAM revealing a case of Hodgkin lymphoma in a 35-year-old patient.

Hemophagocytic lymphohistiocytosis: epidemiological, clinical and biological profile

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.