Shustik C, Bergsagel D E, Pruzanski W
Blood. 1976 Jul;48(1):41-51.
Ninety-seven patients with light chain disease (LCD) were studied. The median survival from diagnosis was 30 mo for 52 patients with kappa-LCD and 10 mo for 45 patients with lambda-LCD (p less than 0.0007). A lower proportion of kappa-LCD patients (15.7%) than lambda-LCD patients (42.2%) died within the first 6 mo after diagnosis. The survival of the remaining patients with kappa-LCD was still much longer than of those with lambda-LCD (p = 0.022). The shorter survival of lambda-LCD patients could not be ascribed to an increased incidence of recognized manifestations indicating a poor prognosis (e.g., anemia, hypercalcemia, azotemia, low albumin, the extent of osteolytic lesions, or proteinuria), the incidence of amyloidosis, the clinical stage of the disease at diagnosis, or the response to treatment, and remains unexplained. A comparison of the clinical manifestations of LCD with those of other myelomas revealed some differences. LCD patients were slightly younger than IgA and IgG patients but older than IgD patients. A 1:1 ratio of males to females was similar to the ratios in IgA and IgG myeloma, but differed from the 3:1 ratio reported for IgD myeloma. Plasma-cell leukemia developed in 7/97 LCD patients, an incidence that was higher than has been reported in other myelomas. The initial BUN was more than or equal to 30 mg/100 ml in 54 of 95 LCD patients, an incidence that was higher than has been reported for IgA and IgG myeloma, but lower than the incidence in IgD myeloma. The incidence of amyloidosis in LCD (23 of 97 patients) was similar to that reported for IgA and IgG myeloma, but less than the incidence in IgD myeloma.
对97例轻链病(LCD)患者进行了研究。52例κ轻链病患者从诊断开始的中位生存期为30个月,45例λ轻链病患者为10个月(p<0.0007)。κ轻链病患者在诊断后前6个月内死亡的比例(15.7%)低于λ轻链病患者(42.2%)。其余κ轻链病患者的生存期仍比λ轻链病患者长得多(p=0.022)。λ轻链病患者生存期较短的原因不能归结于提示预后不良的公认表现(如贫血、高钙血症、氮质血症、低白蛋白、溶骨性病变范围或蛋白尿)发生率增加、淀粉样变性发生率、诊断时疾病的临床分期或对治疗的反应,目前仍无法解释。将轻链病的临床表现与其他骨髓瘤的临床表现进行比较发现了一些差异。轻链病患者比IgA和IgG骨髓瘤患者略年轻,但比IgD骨髓瘤患者年龄大。男女比例为1:1,与IgA和IgG骨髓瘤相似,但与报道的IgD骨髓瘤3:1的比例不同。97例轻链病患者中有7例发生浆细胞白血病,这一发生率高于其他骨髓瘤的报道。95例轻链病患者中有54例初始血尿素氮≥30mg/100ml,这一发生率高于IgA和IgG骨髓瘤的报道,但低于IgD骨髓瘤。轻链病中淀粉样变性的发生率(97例患者中有23例)与IgA和IgG骨髓瘤报道的相似,但低于IgD骨髓瘤。