title: Visceral leishmaniasis associated hemophagocytic lymphohistiocytosis--case report and systematic review.
abstract: BACKGROUND
  The clinical features of leishmaniasis overlap with that of hemophagocytic lymphohistiocytosis (HLH) and the diagnosis of visceral leishmaniasis (VL) related HLH can be challenging.
  
  
  OBJECTIVE
  To review information available on disease course, treatment, adjunctive therapy used and the outcomes of VL related HLH.
  
  
  METHODS
  We describe an illustrative case and review all reported cases of VL associated HLH in the English literature till March 2007.
  
  
  RESULTS
  VL associated HLH is rare, with 56 cases reported in the English literature. Clinical features lack discriminating value to recognize VL as the inciting etiology. Bone marrow aspiration (BMA) establishes the diagnosis in 78% of cases but is often negative at onset of the syndrome due to the pauci-microbial nature of the disease and patchy involvement. Repeated marrow aspiration, liver biopsy, blood cultures and serology may be required to establish the diagnosis. Liposomal amphotericin is the drug of choice. IVIG may be considered when there is an inadequate response to anti-leishmanial therapy in severe and refractory disease.
  
  
  CONCLUSIONS
  VL related HLH is often under-recognized because of overlapping clinical features and negative marrow evaluation at onset, leading to high mortality rates.
authors.affiliation: Department of Pulmonary & Critical Care Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India. visitsrinivasan@gmail.com
authors.forename: Srinivas
authors.initials: S
authors.lastname: Rajagopala
authors.forename: Usha
authors.initials: U
authors.lastname: Dutta
authors.forename: K S Poorna
authors.initials: KS
authors.lastname: Chandra
authors.forename: Prateek
authors.initials: P
authors.lastname: Bhatia
authors.forename: Neelam
authors.initials: N
authors.lastname: Varma
authors.forename: Rakesh
authors.initials: R
authors.lastname: Kochhar
mesh_terms: D000293:Adolescent; D000328:Adult; D000666:Amphotericin B / Q000627:therapeutic use; D000981:Antiprotozoal Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D007898:Leishmaniasis, Visceral / Q000150:complications* / Q000188:drug therapy / Q000469:parasitology; D008081:Liposomes / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D008875:Middle Aged
chemical_list: D000981:Antiprotozoal Agents; D016756:Immunoglobulins, Intravenous; D008081:Liposomes; D000666:Amphotericin B