title: Hemophagocytic lymphohistiocytosis: an unusual complication of leprosy.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  A high index of suspicion is essential for prompt diagnosis of hemophagocytic syndrome in the setting of disseminated infection such as leprosy.
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Wafa
authors.initials: W
authors.lastname: Saidi
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Rima
authors.initials: R
authors.lastname: Gammoudi
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Mouna
authors.initials: M
authors.lastname: Korbi
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Amina
authors.initials: A
authors.lastname: Aounallah
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Lobna
authors.initials: L
authors.lastname: Boussofara
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Najet
authors.initials: N
authors.lastname: Ghariani
authors.affiliation: Department of Pathology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Badreddine
authors.initials: B
authors.lastname: Sriha
authors.affiliation: Department of Hematology Laboratory, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Nejia
authors.initials: N
authors.lastname: Braham
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Denguezli
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Colandane
authors.initials: C
authors.lastname: Belajouza
authors.affiliation: Department of Dermatology, Farhat Hached Hospital, Sousse, Tunisia.
authors.forename: Rafia
authors.initials: R
authors.lastname: Nouira
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D006801:Humans; D007917:Leprostatic Agents / Q000627:therapeutic use; D015440:Leprosy, Lepromatous / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D056164:Teaching Rounds; D016896:Treatment Outcome; D014416:Tunisia
chemical_list: D000305:Adrenal Cortex Hormones; D007917:Leprostatic Agents