[Hemophagocytic syndrome: report of 4 cases].
BACKGROUND
Hemophagocytic syndrome (HPS) is a rare but potentially fatal disease. The diagnosis is based on the combination of clinical and biological signs, requiring histological or cytological research hemophagocytosis and exhaustive etiological investigation.


OBJECTIVE
To report four cases of the HPS in an internal medicine department.


METHODS
We report four cases of HPS associated with Still's disease in two cases, with Sjogren syndrome in one case and a severe sepsis in one case. There are three women and one man. The mean age was 34.75 years, with extremes of 21 to 50 years. In all cases, patients were hospitalized for fever of unknown origin. The etiologic research of this fever remained negative. In all cases, patients validated criteria of HPS confirmed by cytological studies. The treatment was based on corticosteroids in all cases and immunosuppressant in one case. The evolution was favorable in two cases and fatal in two cases.


CONCLUSIONS
HPS is a serious, often overlooked, may affect the prognosis and complicating various infectious, neoplastic or autoimmune diseases.
Hopital Habib Thameur, Tunis, Tunisie.
Besma
B
Ben Dhaou Hmaidi
Fatma
F
Derbali
Fatma
F
Boussema
Sonia
S
Ketari Jamoussi
Lilia
L
Baili
Samir
S
Kochbati
Ouahida
O
Cherif
Lilia
L
Rokbani
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005260:Female; D005335:Fever of Unknown Origin / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D008875:Middle Aged
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents