论文部分内容阅读
迄今世界文献已报道70多例γ重链病(GHCD),仅3例有溶骨损害,其中2例为局限的;而本文报告的1例有广泛性溶骨损害及多发的病理骨折;患者女,34岁:因乏力、消瘦、盗汗3月,加重并高热、咳嗽、胸腰痛,呼吸困难入院,胸腹皮肤有红斑,口腔粘膜及舌有溃疡,腭水肿,全身性淋巴结肿大,肝脾均于肋下2 cm,Hb及BPC数正常。WBC 11.3×10~9/L;分类见非典型嗜碱淋巴细胞占8%;淋巴样浆细胞2%,浆细胞6%;胞核有切迹或分叶,血沉44mm/h,血钙3.38m mol/L(正常值2.05-2.45),磷0.58m mol/L(0.8-1.45);LDH835u/L(228-475),AKP198u/L(15-70)。肝肾功能及其他血生化正常。血清IgA、IgM、IgD、IgE和完整的IgG量正常。肋骨、锁骨、肩胛骨、近端肱骨,股骨,胫骨、脊柱和骨盆X线片均有溶骨灶,颅骨呈穿凿样变,3条肋有病理性骨折,骨髓中非典型嗜碱淋巴细胞占(?).6%,淋巴样浆细胞4.4%,浆细胞15.6%;核形同外
So far in the world literature has reported more than 70 cases of γ heavy chain disease (GHCD), only 3 cases of osteolytic lesions, of which 2 were limited; and the paper reported in 1 case of extensive osteolytic lesions and multiple pathological fractures; patients Female, 34 years old: due to fatigue, weight loss, night sweats in March, aggravating and fever, cough, chest pain, dyspnea hospitalization, chest and abdomen skin erythema, oral mucosa and tongue ulcers, palatal edema, generalized lymphadenopathy, liver Spleen are in the ribs 2 cm, Hb and BPC number normal. WBC 11.3 × 10 ~ 9 / L; classification see atypical alkaloid lymphocytes accounted for 8%; lymphoid plasma cells 2%, plasma cells 6%; nuclear notch or lobulation, erythrocyte sedimentation rate 44mm / h, serum calcium 3.38 m mol / L (normal value 2.05-2.45), phosphorus 0.58m mol / L (0.8-1.45), LDH835u / L (228-475) and AKP198u / L (15-70). Liver and kidney function and other blood biochemical normal. Serum IgA, IgM, IgD, IgE and intact IgG levels were normal. Rib, clavicle, scapula, proximal humerus, femur, tibia, spine and pelvis X-ray films have osteolytic lesions, the skull was wearing chisel-like change, three ribs with pathological fractures, bone marrow atypical basophilic lymphocytes accounted for ?) 6%, lymphoid plasma cells 4.4%, plasma cells 15.6%; nucleus with the same