Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, Japan.
Acta Haematol. 2012;128(3):139-43. doi: 10.1159/000339097. Epub 2012 Aug 10.
An 84-year-old Japanese man was admitted because of pancytopenia. The bone marrow was hypoplastic with a predominance of abnormal small lymphocytes and grape cells, which were positive for CD19 and CD20, and partially for the surface ĸ-light chain. Systemic CT scanning showed neither lymph node swelling nor hepatosplenomegaly. Serum immunoelectrophoresis and rocket immunoselection assays showed the presence of monoclonal IgG protein without a corresponding light chain and faint IgMĸ monoclonal protein. Histologic analysis of the clot preparation of the bone marrow aspirate facilitated a diagnosis of lymphoplasmacytic lymphoma (LPL). PCR analysis of the marrow cells demonstrated a clonal rearrangement of the immunoglobulin heavy-chain gene. From these results, we made a final diagnosis of γ-heavy-chain disease (γ-HCD) with underlying LPL localized in the bone marrow. We performed only a single course of immunochemotherapy (rituximab and fludarabine) in view of severely impaired hematopoiesis, which resulted in marked reduction of lymphoma cells and improvement of hematopoiesis. This report suggests the efficacy of rituximab plus fludarabine therapy for LPL-associated γ-HCD.
一位 84 岁的日本男性因全血细胞减少入院。骨髓增生低下，以异常小淋巴细胞和葡萄细胞为主，这些细胞 CD19 和 CD20 阳性，部分表面 κ 轻链阳性。全身 CT 扫描未见淋巴结肿大或肝脾肿大。血清免疫电泳和火箭免疫电泳检测显示存在单克隆 IgG 蛋白，无相应轻链，微弱 IgMκ 单克隆蛋白。骨髓抽吸物血凝块组织学分析有助于诊断为淋巴浆细胞淋巴瘤（LPL）。骨髓细胞的 PCR 分析显示免疫球蛋白重链基因呈克隆性重排。根据这些结果，我们最终诊断为骨髓中局限性 γ-重链病（γ-HCD）伴 LPL。鉴于造血严重受损，我们仅进行了一个疗程的免疫化疗（利妥昔单抗和氟达拉滨），导致淋巴瘤细胞明显减少，造血功能改善。该报告提示利妥昔单抗联合氟达拉滨治疗 LPL 相关 γ-HCD 的疗效。
