title: [Macrophage activation syndrome and autoimmunity due to visceral leishmaniasis].
abstract: Hemophagocytic syndromes are a heterogeneous group of diseases characterized by an excessive immune response, mediated by activated cytotoxic T cells and macrophages. Among hemophagocytic syndromes, genetic and secondary forms can be distinguished. We report on the case of a male newborn who presented with macrophage activation syndrome associated with lymphoproliferation with favorable outcome under prednisone and cyclosporin. Hemopathy, infection, or genetic lymphohistiocytosis were initially ruled out. Severe autoimmunity was suspected because of positive antinuclear antibodies and Farr test associated with anemia and a positive Coombs test as well as cytolytic hepatitis with anti-liver, kidney microsome (LKM) antibodies. Treatment was therefore intensified by adding mycophenolate mofetil. This led to an unexpected deterioration of general health and lab exam results with recurrence of fever and inflammation. The initial investigations were revisited and completed by a liver biopsy, which revealed the presence of numerous leishmania parasites at the amastigote stage, enabling the diagnosis of visceral leishmaniasis. The patient's condition dramatically improved under liposomal amphotericin B treatment. Our observation shows that visceral leishmaniasis can present as lupus-like syndrome with lymphoproliferation. Moreover, the lack of leishmania on marrow aspiration cannot rule out the diagnosis of visceral leishmaniasis. Detection of leishmania by serological or molecular tests is mandatory in case of hepatosplenomegaly with hemophagocytic syndrome together with autoantibodies, in order to avoid useless and life-threatening exposure to immunosuppressive treatments.
authors.affiliation: Service de pédiatrie, hôpital de Hautepierre, avenue Molière, 67000 Strasbourg cedex, France. Electronic address: higellaetitia@gmail.com.
authors.forename: L
authors.initials: L
authors.lastname: Higel
authors.affiliation: Service d'hématologie cellulaire, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France.
authors.forename: C
authors.initials: C
authors.lastname: Froehlich
authors.affiliation: Service d'hématologie cellulaire, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France.
authors.forename: M-P
authors.initials: MP
authors.lastname: Pages
authors.affiliation: Service d'hématologie cellulaire, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France.
authors.forename: D
authors.initials: D
authors.lastname: Dupont
authors.affiliation: Institut de parasitologie et mycologie médicale, hospices civils de Lyon, centre de biologie Nord, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.
authors.forename: S
authors.initials: S
authors.lastname: Collardeau-Frachon
authors.affiliation: Institut de parasitologie et mycologie médicale, hospices civils de Lyon, centre de biologie Nord, 103, grande rue de la Croix-Rousse, 69004 Lyon, France.
authors.forename: F
authors.initials: F
authors.lastname: Dijoud
authors.affiliation: Service de néphrologie, rhumatologie et dermatologie pédiatrique, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France.
authors.forename: P
authors.initials: P
authors.lastname: Cochat
authors.affiliation: Service de néphrologie, rhumatologie et dermatologie pédiatrique, hôpital Femme-Mère-Enfant, 59, boulevard Pinel, 69677 Bron cedex, France.
authors.forename: A
authors.initials: A
authors.lastname: Belot
mesh_terms: D015551:Autoimmunity; D006801:Humans; D007223:Infant; D007898:Leishmaniasis, Visceral / Q000150:complications*; D055501:Macrophage Activation Syndrome / Q000469:parasitology*; D008297:Male