Validation of the composite organ and hematologic response model for prognostic prediction in a Chinese light chain amyloidosis cohort

Abstract

To achieve prompt and comprehensive assessment of treatment, we investigated the feasibility of composite hematologic and organ response (CHOR) model in a Chinese light chain (AL) amyloidosis cohort. Three hundred and eighty-eight newly diagnosed patients were assigned scores of 0–3 for complete response, very good partial response, partial response, no response, or progression at 6 months. Organ response (OR) was scored as follows: 0 – all OR (AOR), 1 – mixed OR (MOR), and 2 – no OR (NOR). Finally, patients were divided into CHOR group 1 (total score 0–3) and group 2 (total score 4–5). The patients who achieved AOR and MOR had similar outcomes, which were much better than those of patients with NOR. Group 1 had significantly better overall survival than group 2 (p< .001). The CHOR model had a significantly higher predictive power of survival than hematologic response and OR. We validated the value of the CHOR model as an early indicator of treatment benefit.

Keywords:

• Light chain amyloidosis
• hematologic response
• organ response
• composite response
• prognosis

Disclosure statement

The authors declare that they have no competing interests.

Additional information

Funding

This work was supported by the National Natural Science Foundation of China [Grant No. 81974011, for LJ]; the Beijing Natural Science Foundation [Grant No. 7182128, for LJ]; the Capital Health Research and Development of Special Fund [Grant No. 2018-2-4015, for LJ]; The CAMS Innovation Fund for Medical Sciences [Grant No. 2016-12M-1-002, for LJ]; and the National Key Research and Development Program of China [Grant No. 2016YFC0901503, for LJ].