Bhutani Divaya, Chakraborty Rajshekhar, Wats Karan, Hughes Michael Sang, Dor Lotan, Sekulic Miroslav, Lentzsch Suzanne
Division of Hematology/Oncology, Columbia University Medical Center, New York, New York, USA.
Division of Cardiology, Columbia University Medical Center, New York, New York, USA.
Eur J Haematol. 2025 Apr;114(4):700-703. doi: 10.1111/ejh.14393. Epub 2025 Feb 5.
Monoclonal immunoglobulin deposition disease (MIDD) is characterized by deposits of intact monoclonal immunoglobulin protein, most commonly in the kidney, but other organs such as the heart can be involved. Patients are treated with plasma cell-directed therapies. Here we describe the first reported case of a patient with refractory MIDD with renal and cardiac involvement who was successfully treated with a BCMA-drected therapy. After achieving a deep hematologic response, the patient achieved a cardiac organ response as well. BCMA-directed therapy can be valuable for patients with refractory monoclonal immunoglobulin deposition disease.
单克隆免疫球蛋白沉积病(MIDD)的特征是完整的单克隆免疫球蛋白蛋白沉积,最常见于肾脏,但心脏等其他器官也可能受累。患者接受针对浆细胞的治疗。在此,我们描述了首例报告的难治性MIDD合并肾脏和心脏受累患者,该患者通过靶向B细胞成熟抗原(BCMA)的治疗获得成功。在实现深度血液学缓解后,患者的心脏器官也出现了缓解。靶向BCMA的治疗对于难治性单克隆免疫球蛋白沉积病患者可能具有重要价值。