Gandara D R, Mackenzie M R
University of California-Davis.
Med Clin North Am. 1988 Sep;72(5):1155-67. doi: 10.1016/s0025-7125(16)30734-9.
With a large segment of the adult population now undergoing routine screening tests on a periodic basis, findings such as rouleaux when the complete blood count is performed or an elevated total protein and globulin fraction on serum chemistries often lead to the performance of a serum protein electrophoresis. When a monoclonal gammopathy is confirmed, the clinician is faced with a broad differential diagnosis that includes a variety of distinct malignant plasma cell disorders and lymphoproliferative diseases, as well as the high incidence of MGUS in the otherwise healthy adult population. Other benign causes of secondary monoclonal gammopathy, such as underlying inflammatory or infectious disorders or drug reactions, may add to the diagnostic dilemma in some patients. By following a systematic plan of laboratory evaluation such as that described here, however, and always keeping the patient's clinical status as a primary focus, the clinician should be able to arrive at a diagnosis and formulate a therapeutic plan in most instances. The most common differential diagnosis, that of MGUS versus PCM, still is difficult in some cases, and it is emphasized that careful follow up over time remains the best method at present for differentiating these two conditions. Once the basic laboratory evaluation of monoclonal gammopathy has been completed, further work-up will need to be individualized. In some cases, the preliminary evaluation will reveal a key feature, such as a monoclonal gammopathy that is IgM, which will lead to a rapid diagnosis of WMG and alert the clinician to investigate other nonroutine aspects of the laboratory evaluation, such as a serum viscosity or specific tests of hemostatic function. In other patients, the initial laboratory evaluation of monoclonal gammopathy may lead to other recommendations, such as lymph node biopsy for evaluation of possible lymphoma, or tissue biopsy to confirm the suspicion of amyloidosis. Overall, the evaluation of monoclonal gammopathy remains a challenging one, but one in which the clinician usually is rewarded with a diagnosis that will allow him to make appropriate management plans for his patient.
由于现在很大一部分成年人口定期接受常规筛查，全血细胞计数时出现缗钱状或血清化学检查中总蛋白和球蛋白分数升高的结果，常常会导致进行血清蛋白电泳。当确诊为单克隆丙种球蛋白病时，临床医生面临着广泛的鉴别诊断，包括各种不同的恶性浆细胞疾病和淋巴增殖性疾病，以及在其他方面健康的成年人群中意义未明的单克隆丙种球蛋白病（MGUS）的高发病率。继发性单克隆丙种球蛋白病的其他良性病因，如潜在的炎症或感染性疾病或药物反应，可能会在一些患者中增加诊断难题。然而，通过遵循本文所述的系统实验室评估计划，并始终将患者的临床状况作为主要关注点，临床医生在大多数情况下应该能够做出诊断并制定治疗计划。最常见的鉴别诊断，即MGUS与多发性骨髓瘤（PCM）的鉴别，在某些情况下仍然困难，并且需要强调的是，随着时间的推移进行仔细随访仍然是目前区分这两种情况的最佳方法。一旦完成了单克隆丙种球蛋白病的基本实验室评估，进一步的检查将需要个体化。在某些情况下，初步评估会揭示一个关键特征，例如IgM型单克隆丙种球蛋白病，这将导致迅速诊断为华氏巨球蛋白血症（WMG），并提醒临床医生调查实验室评估的其他非常规方面，如血清粘度或止血功能的特定测试。在其他患者中，单克隆丙种球蛋白病的初始实验室评估可能会导致其他建议，如进行淋巴结活检以评估可能的淋巴瘤，或进行组织活检以证实对淀粉样变性的怀疑。总体而言，单克隆丙种球蛋白病的评估仍然具有挑战性，但临床医生通常会因做出诊断而能够为患者制定适当的管理计划而有所收获。
