TY - JOUR
T1 - Hemophagocytic lymphohistiocytosis associated with dermatomyositis
T2 - A case report
AU - Thomas, Aiswarya
AU - Appiah, Juliet
AU - Langsam, Jeffrey
AU - Parker, Susan
AU - Christian, Cecil
PY - 2013/9/1
Y1 - 2013/9/1
N2 - Introduction: 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. Case presentation: A 61-year-old male presented with proximal weakness, rash, periorbital edema, and elevated creatine kinase (CK). He also had pancytopenia, abnormal liverfunctions, 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. Conclusion: 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.
AB - Introduction: 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. Case presentation: A 61-year-old male presented with proximal weakness, rash, periorbital edema, and elevated creatine kinase (CK). He also had pancytopenia, abnormal liverfunctions, 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. Conclusion: 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.
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M3 - Article
C2 - 24156177
AN - SCOPUS:84885223036
SN - 0010-6178
VL - 77
SP - 481
EP - 485
JO - Connecticut Medicine
JF - Connecticut Medicine
IS - 8
ER -