Preud'homme J L, Brouet J C, Seligmann M
Clin Exp Immunol. 1979 Aug;37(2):283-91.
Proliferating cells from twenty-four patients with alpha- or gamma-heavy chain disease (HCD) were studied by direct immunofluorescence and in several cases by biosynthesis experiments with 14C-amino acid incorporation. In twenty-two patients, the cells contained the HCD proteins only and no light chain synthesis could be detected. Conversely, apparently non-secreted monotypic light chains were found in one case of gamma-HCD and one case of alpha-HCD. The proportion of proliferating cells containing cytoplasmic heavy chains, their appearance and the presence or not of surface heavy chains showed great variation from patient to patient. In some cases, the proliferation predominantly affected either plasma cells or lymphocytes whereas in others the disease seemed to correspond to a proliferation of HCD protein-bearing lymphocytes with persistent maturation into plasma cells. Large cell lymphomas supervening on alpha-HCD belonged to the same proliferating clone as the clone secreting the HCD protein, as shown by surface markers and biosynthesis experiments which demonstrated synthesis but no secretion of HCD proteins. In one patient with gamma-HCD, the cells carried surface gamma and delta chains.
通过直接免疫荧光法对24例α或γ重链病(HCD)患者的增殖细胞进行了研究,并在几例患者中通过14C-氨基酸掺入的生物合成实验进行了研究。在22例患者中,细胞仅含有HCD蛋白,未检测到轻链合成。相反,在1例γ-HCD和1例α-HCD患者中发现了明显非分泌型的单型轻链。含有细胞质重链的增殖细胞比例、其外观以及表面重链的有无在患者之间差异很大。在某些情况下,增殖主要影响浆细胞或淋巴细胞,而在其他情况下,疾病似乎对应于携带HCD蛋白的淋巴细胞增殖,并持续成熟为浆细胞。继发于α-HCD的大细胞淋巴瘤与分泌HCD蛋白的克隆属于同一增殖克隆,表面标志物和生物合成实验表明,这些实验证明了HCD蛋白的合成但未分泌。在1例γ-HCD患者中,细胞携带表面γ和δ链。