Zhou Hebing, Chen Wenming, Zhang Juan, Zeng Hui, Jian Yuan, Fu Chenxiao
Department of Hematology, Luhe Hospital, Capital Medical University, Beijing 101149, P.R. China.
Department of Hematology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100021, P.R. China.
Oncol Lett. 2016 Jun;11(6):4147-4151. doi: 10.3892/ol.2016.4515. Epub 2016 May 4.
Heavy chain diseases (HCDs) are rare B cell lymphoplasma cell proliferative disorders that are characterized by the production of incomplete monoclonal immunoglobulin (Ig) heavy chains without the associated light chains. γ-HCD (IgG subtype) is a rare subtype, with ~150 cases reported in the literature to date; however, to the best of our knowledge, no reports of T cell receptor (TCR) gene rearrangement in γ-HCD exist in the literature. The present study reports the case of an 81-year-old man with γ-heavy chain disease associated with TCR gene rearrangement, identified in lymph node biopsy and bone marrow aspirate specimens. The present case revealed an alternative manifestation of γ-HCD, which may provide additional biological insights into this rare B cell disorder.
重链病(HCDs)是罕见的B细胞淋巴浆细胞增殖性疾病,其特征是产生不完整的单克隆免疫球蛋白(Ig)重链而无相关轻链。γ-HCD(IgG亚型)是一种罕见的亚型,迄今为止文献报道约150例;然而,据我们所知,文献中尚无γ-HCD中T细胞受体(TCR)基因重排的报道。本研究报告了1例81岁男性γ重链病合并TCR基因重排的病例,该病例在淋巴结活检和骨髓穿刺标本中得以确诊。本病例揭示了γ-HCD的另一种表现形式,这可能为这种罕见的B细胞疾病提供更多生物学见解。