The impact of chemoimmunotherapy on primary cold agglutinin disease and Waldenström macroglobulinemia-associated cold agglutinin syndrome
All patients (5/5) achieved at least CAH‑PR (ORR 100%).
The study retrospectively evaluated 39 patients with cold agglutinin-associated hemolysis (pCAD, n=5; WM‑CAS, n=5) and Waldenström macroglobulinemia without cold agglutinin syndrome (WM‑only, n=29). Rituximab‑based chemoimmunotherapy (R‑CHOP or bendamustine‑rituximab) was the predominant first‑line treatment. All pCAD patients achieved at least a partial response under CAH criteria (overall response rate, 100 %), with 80 % of WM‑CAS patients achieving a partial response (overall response rate, 80 %). Tissues were molecularly characterized for MYD88 L265P, which was absent in pCAD, present in 1/4 WM‑CAS cases, and detected in 27/29 WM‑only cases.
This sentence demonstrates the complete efficacy of rituximab‑based chemoimmunotherapy in primary cold agglutinin disease patients, highlighting a 100 % overall response rate prognostic for clinical decision‑making in this rare hemolytic disorder.
Evidence level: Számítógépes vagy elméleti. Modellből vagy adatbányászatból származó jel.
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