Treatment of hemophagocytic lymphohistiocytosis with alemtuzumab in systemic lupus erythematosus

Michael P. Keith*, Clovis Pitchford, Wendy B. Bernstein

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

19 Scopus citations

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an immune disorder characterized by cytokine dysregulation and uncontrolled activation of T lymphocytes and macrophages. It is categorized as primary when associated with specific genetic mutations or secondary when associated with infections, malignancies, or autoimmune disorders. Clinical features ofHLHinclude unexplained fever, hepatosplenomegaly, pancytopenia, and severe hyperferritinemia. Treatment of primary HLH has become standardized based on the HLH-2004 protocol using cyclosporine, etoposide, and dexamethasone with or without intrathecal methotrexate followed by hematopoietic stem cell transplantation. Treatment of secondary HLH is directed at control of the underlying condition. If unsuccessful, cytotoxic agents such as those in HLH-2004, steroids, intravenous F-globulin, or targeted immune therapy have been used. Immunotherapy targeting CD52 expressed on immune effector cells of HLH is a rational therapeutic approach in patients too ill for traditional cytotoxic chemotherapy. We describe the successful use of alemtuzumab to treat HLH due to systemic lupus erythematosus.

Original languageEnglish
Pages (from-to)134-137
Number of pages4
JournalJournal of Clinical Rheumatology
Volume18
Issue number3
DOIs
StatePublished - Apr 2012
Externally publishedYes

Keywords

  • Alemtuzumab
  • HLH-2004
  • Hemophagocytic lymphohistiocytosis
  • Macrophage activation syndrome
  • Systemic lupus erythematosus

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