01984nas a2200253 4500000000100000008004100001653000800042653003900050100001200089700001500101700001200116700001600128700001700144700001500161700001200176700001300188700001600201700001600217700001300233245007700246050001400323520137900337022001401716 2015 d10aHLH10aHemophagocytic lymphohistiocytosis1 aSaidi W1 aGammoudi R1 aKorbi M1 aAounallah A1 aBoussofara L1 aGhariani N1 aSriha B1 aBraham N1 aDenguezli M1 aBelajouza C1 aNouira R00aHemophagocytic lymphohistiocytosis : an unusual complication of leprosy. aSAIDI20153 a

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition of chaotic uncontrolled immune system stimulation and not fully understood pathophysiology. Most reported cases of hemophagocytic syndrome in patients with mycobacterial infections have been associated with Mycobacterium tuberculosis. As far as we could ascertain, to date, no established HLH case complicating leprosy has been published in the medical literature.

CASE REPORT: We describe here a new case of Hansen's disease in a 58-year-old Tunisian man with an unusual complicated clinical course documented as hemophagocytic syndrome. Cutaneous and neurological involvements were the main clinical signs of Hansen's disease. Histological findings suggested the diagnosis of leprosy and were somewhat more characteristic of the lepromatous leprosy type. While on antileprosy treatment, he developed unexplained persistent fever, organomegaly, bicytopenia, and elevated rate of inflammatory markers with bone marrow aspirate showing large macrophages with increased phagocytosis of mature and immature blood elements, typical features of hemophagocytic syndrome.

CONCLUSION: A high index of suspicion is essential for prompt diagnosis of hemophagocytic syndrome in the setting of disseminated infection such as leprosy.

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