Ibrutinib‐associated hemophagocytic lymphohistiocytosis: A case series from Johns Hopkins

Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening 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.

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

Zoref-Lorenz

Yuklea

Topaz

et al. 2022

J GEN INTERN MED

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Multiple drugs

2019

Reactions Weekly

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Ibrutinib plus rituximab for the treatment of adult patients with Waldenström’s macroglobulinemia: a safety evaluation

Migkou

Fotiou

Gavriatopoulou

et al. 2021

Expert Opinion on Drug Safety

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Ibrutinib‐associated hemophagocytic lymphohistiocytosis: A case series from Johns Hopkins

Cited by 5 publications

References 14 publications

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

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

Multiple drugs

Ibrutinib plus rituximab for the treatment of adult patients with Waldenström’s macroglobulinemia: a safety evaluation

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