title: Hemophagocytic lymphohistiocytosis associated with dermatomyositis: a case report.
abstract: BACKGROUND
  Hemophagocytic lymphohistiocytosis (HLH) is an uncommon life-threatening disorder of pathologic immune activation. HLH associated with autoimmune disease is recognized although the mechanism remains uncertain. HLH associated with dermatomyositis is very rare and just as rarely reported in medical literature. A high index of suspicion is warranted in the setting of fever, cytopenias, abnormal liver function, and markedly elevated ferritin.
  
  
  METHODS
  A 61-year-old male presented with proximal weakness, rash, periorbital edema, and elevated creatine kinase (CK). He also had pancytopenia, abnormal liver functions, and elevated ferritin. His bone marrow biopsy confirmed HLH. He was treated with high-dose dexamethasone and etoposide and seemed to have been recovering. Soon after his second infusion, he died of a complication from an unrelated endoscopic procedure.
  
  
  CONCLUSIONS
  Early recognition of HLH is critical to the establishment of appropriate management and the prevention of fatal outcomes. We should be aware of the cardinal signs and symptoms of HLH and it should be considered in patients presenting with inflammatory myopathies associated with cytopenias, abnormal liver functions, and markedly elevated ferritin level.
authors.affiliation: Department of Primary Care Internal Medicine, University of Connecticut Medical Center, Farmington, USA. Thomas.aiswarya@gmail.com
authors.forename: Aiswarya
authors.initials: A
authors.lastname: Thomas
authors.forename: Juliet
authors.initials: J
authors.lastname: Appiah
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Langsam
authors.forename: Susan
authors.initials: S
authors.lastname: Parker
authors.forename: Cecil
authors.initials: C
authors.lastname: Christian
mesh_terms: D003882:Dermatomyositis / Q000150:complications* / Q000175:diagnosis*; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged