# A Case of Cold Agglutinin Disease With Transformation to High-Grade Lymphoma During Sutimlimab Treatment

**Authors:** Shota Yamaguchi, Kenki Saito, Keiji Shimada, Naoya Kaneko

PMC · DOI: 10.7759/cureus.87006 · Cureus · 2025-06-29

## TL;DR

A patient with cold agglutinin disease developed aggressive lymphoma during treatment, showing the need for close monitoring and bone marrow evaluation.

## Contribution

Reports a rare case of CAD transforming into high-grade lymphoma during sutimlimab therapy, emphasizing the need for BMB reassessment.

## Key findings

- Sutimlimab initially improved anemia in a CAD patient.
- Bone marrow biopsy revealed transformation to aggressive B-cell lymphoma.
- Chemotherapy led to further improvement in anemia after lymphoma diagnosis.

## Abstract

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia characterized by monoclonal immunoglobulin M-mediated cold agglutinins and monoclonal B-cell proliferation. Sutimlimab, a complement C1s inhibitor, alleviates hemolytic anemia in CAD by blocking the classical complement pathway. We report a case of a 67-year-old Japanese woman with CAD who experienced temporal improvement in anemia following sutimlimab treatment. 18F-fluorodeoxyglucose positron-emission tomography/computed tomography and bone marrow biopsy (BMB) revealed histological transformation into aggressive B-cell lymphoma. Subsequent chemotherapy with polatuzumab vedotin, rituximab, cyclophosphamide, doxorubicin, and prednisolone resulted in further improvement in anemia. This case highlights the importance of reassessing underlying conditions through BMB in cases where sutimlimab treatment is ineffective.

## Linked entities

- **Chemicals:** 18F-fluorodeoxyglucose (PubChem CID 68614)
- **Diseases:** cold agglutinin disease (MONDO:0018922), autoimmune hemolytic anemia (MONDO:0020108)
- **Species:** Homo sapiens (taxon 9606)

## Full-text entities

- **Genes:** C1S (complement C1s) [NCBI Gene 716] {aka EDSPD2}, SLC17A5 (solute carrier family 17 member 5) [NCBI Gene 26503] {aka AST, ISSD, NSD, SD, SIALIN, SIASD}, CD79A (CD79a molecule) [NCBI Gene 973] {aka IGA, IGAlpha, MB-1, MB1}, ALB (albumin) [NCBI Gene 213] {aka FDAHT, HSA, PRO0883, PRO0903, PRO1341}, HP (haptoglobin) [NCBI Gene 3240] {aka HP2ALPHA2, HPA1S}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, FGB (fibrinogen beta chain) [NCBI Gene 2244] {aka HEL-S-78p}
- **Diseases:** bleeding (MESH:D006470), B-LPD (MESH:D015448), macrocytic anemia (MESH:D000748), fatigue (MESH:D005221), hypertension (MESH:D006973), epigastric abdominal pain (MESH:D015746), hemolytic anemia (MESH:D000743), lymphadenopathy (MESH:D008206), fibrosis (MESH:D005355), mCR (MESH:D012075), CAD (MESH:D000744), macrocytosis (MESH:C564004), Tumors (MESH:D009369), infections (MESH:D007239), chronic lymphocytic leukemia (MESH:D015451), splenomegaly (MESH:D013163), hemolysis (MESH:D006461), Lymphoma (MESH:D008223), Anemia (MESH:D000740), B-cell lymphoma (MESH:D016393), diffuse large B-cell lymphoma (MESH:D016403)
- **Chemicals:** creatinine (MESH:D003404), cyclophosphamide (MESH:D003520), Cre (-), 18F-FDG (MESH:D019788), polatuzumab vedotin (MESH:C000600736), Fe (MESH:D007501), thyroxine (MESH:D013974), folic acid (MESH:D005492), hematoxylin (MESH:D006416), doxorubicin (MESH:D004317), uric acid (MESH:D014527), Zn (MESH:D015032), CHP (MESH:C048279), cyclophosphamide, doxorubicin, (MESH:C038334), prednisolone (MESH:D011239), copper (MESH:D003300), vitamin B12 (MESH:D014805), Sutimlimab (MESH:C000634098), H&amp;E (MESH:D006371), rituximab (MESH:D000069283), bendamustine (MESH:D000069461), eosin (MESH:D004801), bilirubin (MESH:D001663)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

7 references — full list in the complete paper: https://tomesphere.com/paper/PMC12308100/full.md

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Source: https://tomesphere.com/paper/PMC12308100