Department of Molecular and Translational Medicine, Faculty of Medicine, University of Brescia, Brescia, Italy.
PLoS One. 2013 Aug 5;8(8):e70811. doi: 10.1371/journal.pone.0070811. Print 2013.
Plasma cell dyscrasias are immunosecretory disorders that can lead to hematological malignancies such as Multiple Myeloma (MM). MM accounts for 15% of all hematologic cancers, and those diagnosed with MM typically become severely ill and have a low life expectancy. Monoclonal immunoglobulin Free Light Chains (FLC) are present in the serum and urine of many patients with plasma cell diseases. The biological differences between monoclonal FLCs, produced under malignant or benign dyscrasias, has not yet been characterized. In the present study, we show that endothelial and heart muscle cell lines internalize kappa and lambda FLCs. After internalization, FLCs are rerouted in the extracellular space via microvesicles and exosomes that can be re-internalized in contiguous cells. Only FLCs secreted from malignant B Lymphocytes were carried in Hsp70, annexin V, and c-src positive vesicles. In both MM and AL Amyloidosis patients we observed an increase in microvesicle and exosome production. Isolated serum vesicles from MM, AL Amyloidosis and monoclonal gammopathy of undetermined significance (MGUS) patients contained FLCs. Furthermore MM and AL amyloidosis vesicles were strongly positive for Hsp70, annexin V, and c-src compared to MGUS and control patients. These are the first data implying that FLCs reroute via microvesicles in the blood stream, and also suggest a potential novel mechanism of c-src activation in plasma cell dyscrasia.
浆细胞异常是免疫分泌紊乱,可以导致血液系统恶性肿瘤,如多发性骨髓瘤(MM)。MM 占所有血液系统癌症的 15%,诊断为 MM 的患者通常病情严重,预期寿命短。单克隆免疫球蛋白游离轻链(FLC)存在于许多浆细胞疾病患者的血清和尿液中。恶性或良性异常产生的单克隆 FLC 之间的生物学差异尚未得到描述。在本研究中,我们表明内皮细胞和心肌细胞系内化κ和λ FLC。内化后,FLC 通过小泡和外泌体在细胞外空间重新途径,这些小泡和外泌体可以被相邻细胞重新内化。只有从恶性 B 淋巴细胞分泌的 FLC 才携带 Hsp70、膜联蛋白 V 和 c-src 阳性囊泡。在 MM 和 AL 淀粉样变性患者中,我们观察到微泡和外泌体产生增加。从 MM、AL 淀粉样变性和意义未明的单克隆丙种球蛋白病(MGUS)患者分离的血清囊泡中含有 FLC。此外,与 MGUS 和对照患者相比,MM 和 AL 淀粉样变性囊泡对 Hsp70、膜联蛋白 V 和 c-src 的阳性率更高。这些是 FLC 通过血流中小泡重新途径的首批数据,也提示浆细胞异常中 c-src 激活的潜在新机制。