Baker A S, Lankford P, Krantz S B, Buchanan R D
South Med J. 1977 Apr;70(4):495-7. doi: 10.1097/00007611-197704000-00040.
A patient with gamma heavy chain disease (Franklin's disease) was discovered during evaluation for pancytopenia and splenomegaly. Lymphadenopathy, palatal edema, and infiltration of the bone marrow palatal edema, and infiltration of the bone marrow with abnormal cells were all absent. Serum and urine protein electrophoresis demonstrated a monoclonal protein migrating in the beta region. Immunoelectrophoresis showed that it reacted with antibodies against the Fc fragment of IgG heavy chains (gamma chains) but not with antibodies against kappa and lambda but not with antibodies against kappa and lambda light chains of Fab fragments. In the first year after detection of the disease, the patient had acute cholecystitis and disseminated herpes zoster. Sixteen months after diagnosis he died of overwhelming pneumonia caused by Pseudomonas aeruginosa and lebsiella neumoniae. A striking feature of his illness was his asymptomatic presentation, with pancytophenia and splenomegaly the only indication of this disease.
一名患有γ重链病（富兰克林病）的患者在全血细胞减少和脾肿大评估过程中被发现。患者不存在淋巴结病、腭部水肿以及骨髓腭部水肿和异常细胞浸润。血清和尿蛋白电泳显示在β区有单克隆蛋白迁移。免疫电泳表明，该蛋白与抗IgG重链（γ链）Fc片段的抗体发生反应，但不与抗Fab片段κ和λ轻链的抗体发生反应。在疾病检测后的第一年，该患者患急性胆囊炎和播散性带状疱疹。诊断16个月后，他死于铜绿假单胞菌和肺炎克雷伯菌引起的重症肺炎。其病情的一个显著特征是无症状表现，全血细胞减少和脾肿大是该疾病的唯一指征。
