title: [Syndrome d'activation macrophagique: une série de 11 cas tunisiens].
abstract: BACKGROUND
  Hemophagocytic syndromes are mostly associated with underlying pathology, they can reveal: immunodeficiency, infections, hemopathies, cancers and auto-immune diseases.
  
  
  OBJECTIVE
  to investigate clinical, biological features, outcome characteristics and underlying pathology of Tunisian patients with hemophagocytic syndromes.
  
  
  METHODS
  A retrospective study of patients with hemophagocytic syndromes admitted in an internal medicine department in Tunis over the period 2009-2012.
  
  
  RESULTS
  There were 11 patients included, 4 men and and 7 women. Mean age was 47,3years. Purpura was observed in 3 cases, hepatosplenomegaly in 6 cases and peripheral lymph nodes in 3 cases. Hemorrhagic complication was noted in 2 cases. There were an increase of inflammatory indices in all the cases. Eight patients had pancytopenia and 3 had bicytopenia. Six patients developed intravascular dissiminated coagulation. High levels of triglyceridemia was noted in 5 cases and an hyperferritinaemia in all cases. Cytological examination of bone marrow confirmed hemophagocytosis in ten cases. Hemophagocytosis was associated to infectious diseases in 6 cases, there were 2 cases of leishmaniasis, septicemia to E.Coli and staphylococcus aureus and lymph nodes tuberculosis with HIV. We also noted a case of lymphoma, myelodysplasic syndrome, rheumatoid polyarthritis, adult onset still's disease. In one patient, the origin of hemophagocytosis remained indefinite. Visceral leishmaniose were treated by Glucantime®, adult Still's disease by corticosteroids and méthrotrexate®, lymphoma by chemotherapy. In lymph nodes tuberculosis, there were good evolution. Six patients died.
  
  
  CONCLUSIONS
  Hemophagocytic syndrome is a rare and life-threatening disease. Pejorative prognosis requires an early therapy with etiological treatment.
authors: Imen
authors: I
authors: Boukhris
authors: Imene
authors: I
authors: Rachdi
authors: Eya
authors: E
authors: Chérif
authors: Samira
authors: S
authors: Azzabi
authors: Lamia
authors: L
authors: Ben Hassine
authors: Iness
authors: I
authors: Kéchaou
authors: Zouleikha
authors: Z
authors: Kaouech
authors: Chékib
authors: C
authors: Kooli
authors: Narjess
authors: N
authors: Khalfallah
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006529:Hepatomegaly / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D055501:Macrophage Activation Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013163:Splenomegaly / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D014416:Tunisia / Q000453:epidemiology; D055815:Young Adult