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Case Reports
. 2024 Oct;15(5):844-850.
doi: 10.14740/wjon1917. Epub 2024 Aug 10.

Secondary Hemophagocytic Lymphohistiocytosis Following Dostarlimab Treatment in a Patient With Metastatic Endometrial Cancer

Emilie Rollet  1 , Sebastien Puigrenier  2 , Abeer Najem  1 , Benoit Desgrousilliers  1 , Pierre Riviere  1
Affiliations
Case Reports

Secondary Hemophagocytic Lymphohistiocytosis Following Dostarlimab Treatment in a Patient With Metastatic Endometrial Cancer

Emilie Rollet et al. World J Oncol. 2024 Oct.

Abstract

Immunotherapy is a rapidly expanding cancer treatment strategy. Dostarlimab is administered as the first-line treatment for metastatic endometrial cancer in combination with chemotherapy. Herein, we describe the case of a 72-year-old female patient who developed hemophagocytic lymphohistiocytosis after receiving a single dose of 500 mg of dostarlimab. The patient's clinical outcome improved after treatment with ruxolitinib and corticosteroids. Oncological treatment was resumed in combination with chemotherapy alone.

Keywords: Adverse effect; Corticosteroids; Endometrial cancer; Hemophagocytic lymphohistiocytosis; Immunotherapy; Ruxolitinib.

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Conflict of interest statement

None to declare.

Figures

Figure 1
Figure 1
Pathophysiology of hemophagocytic lymphohistiocytosis (adapted from [2]). Primary HLH involves susceptibility genes necessary for the functioning of the immune system, whether it be related to autoinflammatory diseases or a deficiency in clearing certain viruses. Additionally, acquired susceptibility can play a role. The secondary mechanism is triggered by factors such as a viral (EBV) or bacterial (Mycobacterium tuberculosis) infection, the use of an immune checkpoint inhibitor, or a malignant hematopathy. Subsequently, there is an inflammation cascade involving antigen-presenting cells and inflammatory cytokines such as IFN-γ or IL-12, leading to hyperactivation of cytotoxic T lymphocytes and macrophages. This hyperactivation is responsible for the various described symptoms. Notably, the hyperactivation of immune cells involves the tyrosine kinases JAK 1 and 2 at the intracellular level. ASD: autoimmune systemic disease; CART: chimeric antigen receptor T-cell therapy; CD163: cluster of differentiation 163; CHS: Chediak-Higashi syndrome; DHS: drug hypersensitivity syndrome; EBV: Epstein-Barr virus; ICI: immune checkpoint inhibitor; IFN-γ: interferon gamma; IL: interleukin; MHC-I: major histocompatibility complex class I; NK: natural killer; sIL-2R: soluble interleukin-2 receptor; TCR: T-cell receptor; TLR: Toll-like receptor; TNF-α: tumor necrosis factor alpha; XLP1: X-linked lymphoproliferative disease 1.
Figure 2
Figure 2
Diffuse morbilliform rash, appearing 14 days after the first treatment.
Figure 3
Figure 3
Chest CT revealed bilateral pleural effusions associated with right lower lobe atelectasis. CT: computed tomography.
Figure 4
Figure 4
Evolution of biological markers after the introduction of ruxolitinib and corticosteroid therapy (ruxolitinib was introduced on January 13, 2024, and corticosteroid therapy on January 14, 2024).
See this image and copyright information in PMC

References

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