Masai R, Wakui H, Togashi M, Maki N, Ohtani H, Komatsuda A, Sawada K
Third Department of Internal Medicine, Akita University School of Medicine, Akita Kumiai General Hospital, Akita, Japan.
Clin Nephrol. 2009 Jan;71(1):9-20. doi: 10.5414/cnp71009.
There are three subtypes of monoclonal immunoglobulin deposition disease: light chain deposition disease (LCDD), light and heavy chain deposition disease (LHCDD), and heavy chain deposition disease (HCDD). Although it has been considered that LHCDD is a variant of LCDD, information on clinicopathological features and prognosis in LHCDD is presently limited.
We reviewed 5,443 renal biopsies, and evaluated clinicopathological features and outcomes in patients with LHCDD, in comparison with those in patients with LCDD and previously reported patients with HCDD. We also characterized paraprotein deposits in patients with LHCDD.
We identified 6 patients with LHCDD, 6 patients with LCDD, and 1 patient with HCDD. The most common clinicopathological findings in patients with LHCDD were proteinuria, renal insufficiency, and nodular sclerosing glomerulopathy. Three patients had IgG-k deposits and 3 patients had IgG-l deposits. Heavy chain subclass analysis performed in 4 patients showed IgG3 deposits in all patients. Dual immunostaining revealed glomerular colocalization of light and heavy chains. In contrast with LCDD, glomerular C3 and C1q deposits were common findings in LHCDD and HCDD. All patients with LHCDD were treated with steroids and cytotoxic agents, but no effect on proteinuria was observed. Three patients developed end-stage renal disease requiring hemodialysis. The underlying hematological disorders in LHCDD and HCDD were milder than in LCDD. Early renal survival and overall patient survival in our patients appeared to be better in LHCDD than in LCDD.
There are apparent differences in clinicopathological features and prognosis between LHCDD and LCDD. LHCDD is probably more similar to HCDD.
单克隆免疫球蛋白沉积病有三种亚型：轻链沉积病（LCDD）、轻链和重链沉积病（LHCDD）以及重链沉积病（HCDD）。尽管LHCDD被认为是LCDD的一种变体，但目前关于LHCDD临床病理特征和预后的信息有限。
我们回顾了5443例肾活检病例，并评估了LHCDD患者的临床病理特征和预后，与LCDD患者及先前报道的HCDD患者进行比较。我们还对LHCDD患者的副蛋白沉积物进行了特征分析。
我们识别出6例LHCDD患者、6例LCDD患者和1例HCDD患者。LHCDD患者最常见的临床病理表现为蛋白尿、肾功能不全和结节性硬化性肾小球病。3例患者有IgG-κ沉积，3例患者有IgG-λ沉积。对4例患者进行的重链亚类分析显示所有患者均有IgG3沉积。双重免疫染色显示轻链和重链在肾小球中共定位。与LCDD不同，肾小球C3和C1q沉积在LHCDD和HCDD中是常见表现。所有LHCDD患者均接受了类固醇和细胞毒性药物治疗，但未观察到对蛋白尿有影响。3例患者发展为需要血液透析的终末期肾病。LHCDD和HCDD中的潜在血液系统疾病比重链沉积病中的轻。我们的患者中，LHCDD的早期肾脏生存率和总体患者生存率似乎比LCDD更好。
LHCDD和LCDD在临床病理特征和预后方面存在明显差异。LHCDD可能与HCDD更相似。
