Abstract

Objective: Macrophage activation syndrome is a rare but potentially fatal condition. The diagnosis is often difficult, because the symptoms are not very specific and can mimic those of sepsis, however, its diagnosis is based on the association of clinical and biological, non-specific signs, coupled with cytological or histological research of hemophagocytosis and an exhaustive etiological investigation. Design and method: We report the case of a 40-year-old man with a history of chronic renal failure of undetermined origin, who received a kidney transplant in 2011 from a living related donor. The clinical objective shows signs of dehydration associated with non-fetid diarrhea with the presence of a tense and sensitive abdomen on palpation evoking a surgical abdomen, all evolving in a febrile context; Haematological involvement such as bicytopenia and a positive CMV PCR. Our patient was operated on urgently for peritonitis, after performing peritoneal cleansing, the surgeon took biopsy samples from the various mesenteric lymph nodes. Results: The diagnosis of Macrophage activation syndrome is made in the face of clinical and biological arguments in favor, the histological study of the mesenteric lymph nodes revealing non-Langerhancan lymph node histiocytosis CD 68 + CD 10 evoking a syndrome of macrophage activation and confirmed by the myelogram Conclusions: Much progress remains to be made in the field of SAM in adults, the severity of its prognosis, essentially in a kidney transplant patient, requires an aggressive diagnostic approach and multidisciplinary therapeutic management.

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