A Case of Chronic Lymphocytic Leukemia Complicated by Hemophagocytic Lymphohistiocytosis: Identifying the Aberrant Immune Response

Adi Zoref-Lorenz, Mona Yuklea, Guy Topaz, Michael B. Jordan, Martin Ellis

Research output: Contribution to journalArticlepeer-review

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that may complicate hematologic malignancies. HLH and malignancies have common clinical features, and HLH diagnostic criteria (HLH-2004/Hscore) were not validated in this specific population. We describe a case of a 72-year-old patient with a history of chronic lymphocytic leukemia stable for over 10 years who presented with fever and cytopenia. After excluding infectious etiologies and the progression of her disease, HLH was diagnosed. The patient was treated with etoposide, dexamethasone, intravenous immunoglobulin, and rituximab. Despite initial clinical improvement, the patient deteriorated and developed pulmonary aspergillosis and CNS involvement that reflected uncontrolled HLH. The patient died 45 days after her presentation. An unusual feature of this case was that HLH was not triggered by infection, disease transformation, or treatment. This case emphasizes the challenges of differentiating the development of overwhelming HLH from other complications associated with hematologic malignancy.

Original languageEnglish
Pages (from-to)1542-1546
Number of pages5
JournalJournal of General Internal Medicine
Volume37
Issue number6
DOIs
StatePublished - May 2022

Keywords

  • CLL
  • HLH
  • chronic lymphocytic leukemia
  • fever
  • hemophagocytic lymphohistiocytosis
  • hemophagocytic syndrome
  • pancytopenia

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