Hemophagocytic lymphohistiocytosis associated with dermatomyositis: A case report

Aiswarya Thomas, Juliet Appiah, Jeffrey Langsam, Susan Parker, Cecil Christian

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)481-485
Number of pages5
JournalConnecticut Medicine
Volume77
Issue number8
StatePublished - Sep 1 2013

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Hemophagocytic Lymphohistiocytosis
Dermatomyositis
Ferritins
Pancytopenia
Myositis
Fatal Outcome
Liver
Immune System Diseases
Etoposide
Creatine Kinase
Exanthema
Dexamethasone
Autoimmune Diseases
Signs and Symptoms
Edema
Fever
Bone Marrow
Biopsy

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Hemophagocytic lymphohistiocytosis associated with dermatomyositis : A case report. / Thomas, Aiswarya; Appiah, Juliet; Langsam, Jeffrey; Parker, Susan; Christian, Cecil.

In: Connecticut Medicine, Vol. 77, No. 8, 01.09.2013, p. 481-485.

Research output: Contribution to journalArticle

Thomas, A, Appiah, J, Langsam, J, Parker, S & Christian, C 2013, 'Hemophagocytic lymphohistiocytosis associated with dermatomyositis: A case report', Connecticut Medicine, vol. 77, no. 8, pp. 481-485.
Thomas, Aiswarya ; Appiah, Juliet ; Langsam, Jeffrey ; Parker, Susan ; Christian, Cecil. / Hemophagocytic lymphohistiocytosis associated with dermatomyositis : A case report. In: Connecticut Medicine. 2013 ; Vol. 77, No. 8. pp. 481-485.
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