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. 2025 Sep 1;110(9):2235-2239.
doi: 10.3324/haematol.2025.287532. Epub 2025 May 15.

Marginal zone lymphoma with anti-factor H IgM and atypical hemolytic uremic syndrome successfully treated with odronextamab

Gianluigi Ardissino  1 , Piera Angelillo  2 , Maria Cristina Mancuso  1 , Samantha Griffini  3 , Elena Grovetti  3 , Luigi Porcaro  4 , Thomas Ria  1 , Sarah Marktel  2 , Flora Peyvandi  5 , Stefano Luminari  6 , Andrés J M Ferreri  2 , Michele Merli  7 , Massimo Cugno  8
Affiliations

Marginal zone lymphoma with anti-factor H IgM and atypical hemolytic uremic syndrome successfully treated with odronextamab

Gianluigi Ardissino et al. Haematologica. .
No abstract available

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Figures

Figure 1.
Figure 1.
Effects of specific monoclonal antibodies, targeted to known epitopes of factor H, on the binding of patient’s autoantibodies to immobilized factor H molecule. The column charts represent the binding of serum IgM anti-factor H autoantibodies to immobilized factor H, expressed as mean optical density (OD) of 3 experiments with standard deviation. In the present patient, the binding was inhibited only by the monoclonal antibody (mAb) L20 that interacts with the SCR domain 19 (the active site of factor H for its binding to endothelial cells), whereas mAb OX23 (interacting with SCR1-4), OX24 (interacting with SCR5), and C18 (interacting with SCR20) did not modify the binding of serum anti-factor H autoantibodies to the factor H molecule. The positive control is a patient with previously documented autoantibodies to factor H that interact with domain 19. The negative control is normal pooled serum.
Figure 2.
Figure 2.
Computed tomography scan of the abdomen and gastroscopy before and after odronextamab therapy. (A) Computed tomography (CT) scan of abdomen with contrast medium performed before odronextamab therapy. The green arrow shows celiac tripod adenopaties. The yellow arrow shows moderate splenomegaly. (B) CT scan of abdomen with contrast medium performed after 3 months of odronextamab therapy showing regression of celiac tripod adenopaties and splenomegaly. (C) Gastroscopy performed before odronextamab therapy. The blue arrow shows a fibrinous ulcer. Gastric biopsy at this site showed clear involvement by marginal zone lymphoma. (D) Gastroscopy performed after 3 months odronextamab therapy showing a complete regression of the ulcer.
Figure 3.
Figure 3.
Time course of total and specific anti-factor H IgM concentrations in patient’s serum. Ibrutinib therapy was administered from November 2022 to January 2024 while odronextamab therapy was started on February 27, 2024 and is still ongoing. Total immunoglobulin (Ig)M normal range: 40-230 mg/dL. Anti-factor H IgM normal range: 0-10.3 AU/mL.
See this image and copyright information in PMC

References

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