Mohan Meera, Gokden Murat, Gokden Neriman, Schinke Carolina
Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Department of Pathology , University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Am J Case Rep. 2016 Mar 18;17:173-6. doi: 10.12659/ajcr.895762.
Light chain deposition disease is a systemic disease characterized by deposition of immunoglobin light chains in various organs. Cardiac involvement of light chain deposition disease, also known as cardiac nonamyloidotic immunoglobin deposition disease (CIDD), is a rare clinical entity, where clinical outcome is very variable and best treatment approaches are not well known.
We present the case of a 31-year-old man with a solitary thoracic plasmacytoma and cardiac light chain deposition disease with evidence of congestive heart failure by echocardiography and cardiac markers. The patient underwent surgical resection of the plasmacytoma followed by systemic therapy with 50% VDT-PACE and then VRD with near-normalization of his heart function. A melphalan-based stem cell transplant is planned in this young patient to achieve the best possible long-term remission.
CIDD is a very rare disease, with previous reports showing diverse manifestations with variable outcome. A high level of clinical suspicion should be maintained in such cases and early intervention, as in our patient, can restore cardiac function. There is very little literature on the optimal management of these patients. A combination of surgery and chemotherapy were pursued in our patient with very good results.
轻链沉积病是一种系统性疾病，其特征为免疫球蛋白轻链在各个器官沉积。轻链沉积病的心脏受累，也称为心脏非淀粉样免疫球蛋白沉积病（CIDD），是一种罕见的临床实体，其临床结局差异很大，最佳治疗方法也尚不明确。
我们报告一例31岁男性患者，患有孤立性胸段浆细胞瘤和心脏轻链沉积病，超声心动图和心脏标志物检查显示有充血性心力衰竭的证据。该患者接受了浆细胞瘤手术切除，随后采用50% VDT-PACE方案进行全身治疗，然后采用VRD方案，心功能接近正常。计划对这位年轻患者进行基于美法仑的干细胞移植，以实现尽可能好的长期缓解。
CIDD是一种非常罕见的疾病，既往报道显示其表现多样，结局各异。对于此类病例应保持高度的临床怀疑，并且如我们的患者一样，早期干预可恢复心功能。关于这些患者的最佳管理的文献非常少。我们的患者采用手术和化疗相结合的方法，取得了非常好的效果。
