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. 2024 Sep 1;109(9):2998-3003.
doi: 10.3324/haematol.2023.284179.

Emapalumab as salvage therapy for adults with malignancy-associated hemophagocytic lymphohistiocytosis

William T Johnson  1 , Zachary D Epstein-Peterson  2 , Nivetha Ganesan  3 , Timothy Pak  4 , Tiffany Chang  3 , Phuong Dao  5 , Alison J Moskowitz  2 , Robert N Stuver  2 , Paola Ghione  2 , Natasha Galasso  3 , Niloufer Khan  6 , M Lia Palomba  2 , Philip C Caron  2 , Anita Kumar  2 , Roni Tamari  7 , Jennifer K Lue  2 , Ariela Noy  2 , Lorenzo Falchi  2 , Andrew M Intlekofer  8 , Boglarka Gyurkocza  7 , Miguel-Angel Perales  7 , Michael Scordo  7 , A Zara Herskovits  9 , Gilles Salles  2 , Santosha A Vardhana  8 , Steven M Horwitz  2
Affiliations

Emapalumab as salvage therapy for adults with malignancy-associated hemophagocytic lymphohistiocytosis

William T Johnson et al. Haematologica. .
No abstract available

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Figures

Figure 1.
Figure 1.
Swimmer plot summarizing the treatment course from day -35 prior to the first dose of emapalumab in 15 patients with malignancy-associated hemophagocytic lymphohistiocytosis. All patients received steroids from the start of malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH) treatment and concurrently with emapalumab. The start of frontline M-HLH-directed therapy included initiation of steroids and/or etoposide. Last malignancy-directed therapy included any treatment other than steroids and/or etoposide alone. Last malignancy-directed therapies prior to day -35 are not shown. ID: identity; EBV: Epstein-Barr virus; DLBCL: diffuse large B-cell lymphoma; MF: mycosis fungoides; PTCL-NOS: peripheral T-cell lymphoma not otherwise specified; THRLBCL: T-cell/histiocyte-rich large B-cell lymphoma; PTLD: post-transplant (allogeneic stem cell transplant) lymphoproliferative disorder; ALK: anaplastic lymphoma kinase; ALCL: anaplastic large cell lymphoma; PTCL-TFH: peripheral T-cell lymphoma with a follicular T-helper cell phenotype; AITL: angioimmunoblastic T-cell lymphoma; WM: Waldenström macroglobulinemia; MDS-EB2: myelodysplastic syndrome with excess blasts 2; HTLV-1: human T-lymphotropic virus type 1; ATLL: adult T-cell leukemia/lymphoma; ENKTL: extranodal NK/T-cell lymphoma.
Figure 2.
Figure 2.
Percent change in malignancy-associated hemophagocytic lymphohistiocytosis biomarkers for patients surviving ≥72 hours after their first emapalumab dose as determined at the time of best ferritin response. Malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH) biomarker responses were not calculated if their corresponding values were not abnormal at the time of the first dose of emapalumab or if they were never evaluated and these represent the missing values. Patients 5 and 11 did not demonstrate a change in soluble interleukin-2 receptor level below the upper limit that is clinically reported (20,000 pg/mL) at any time point. sIL2r: soluble interleukin-2 receptor α; LDH: lactate dehydrogenase; AST: aspartate aminotransferase; ALT: alanine aminotransferase.
Figure 3.
Figure 3.
Longitudinal improvements in malignancy-associated hemophagocytic lymphohistiocytosis biomarkers in three patients who demonstrated both a >50% reduction as their best ferritin response, and a sustained improvement in soluble interleukin-2 receptor α with emapalumab monotherapy after progression on etoposide and steroids. Percent change at each timepoint was calculated from day -6 before emapalumab for patients 3 and 8, and from day -7 before emapalumab for patient 13. AST: aspartate aminotransferase; ALT: alanine aminotransferase; Tbili: total bilirubin; sIL2r: soluble interleukin-2 receptor α; LDH: lactate dehydrogenase; ANC: absolute neutrophil count; ALC: absolute lymphocyte count.
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References

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